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204 Cards in this Set
- Front
- Back
- 3rd side (hint)
Glycogen regulation by insulin and glucogon/epinephrine diagram |
Back (Definition) |
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Cytoplasm metabolic site |
1- Fatty acid synthesis 2- Protein synthesis 3- Nucleotide 4-Synthesis of cholesterol (SER) 5-HMP shunt 6-Glycolysis |
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Metabolic site both mitochondria and cytoplasm |
1- Heme synthesis 2- Urea cycle 3- Gluconeogenesis |
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Kinase |
Catalysis the transfer of a phosphate group from a high energy molecule (ATP) to a substrate |
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Phophorylase |
Adds inorganic phosphate group to a substrate without the use of ATP |
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Phosphate |
Remove phosphate group from a substrate |
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Dehydrogenase |
Catalyzes oxidation/reduction reactions |
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Carboxylase |
Transfer CO group to a molecule with the help of biotin |
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Hydroxylase |
Add hydroxyl OH group to a substrate |
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Mutase |
Relocated functional groups within a molecule |
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Synthase/synthetase(use ATP or GTP) |
Joint two molecules together using a source of energy |
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De carboxylase |
Removal of a CO group from a substrate |
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Rate determining enzyme of glycolysis gluconeogenesis TCA |
Glycolysis 1- Phosphofructokinase 1 (PFK-1) + AMP fructose 2,6 biphosphate - ATP citrate Gluconeogenesis 1- Fructose 1,6 biphosphate - AMP fructose 2,6 biphosphate TCA 1- Isocitrate dehydrogenase + ADP - ATP NADH |
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Rate determining enzymes for Glycogenesis Glycogenlysis HMP shunt |
Glucogenesis 1- Glycogen synthase + G6P, insulin cortisol - epinephrine glucogan Glycogenlysis 1- Glycogen phosphorylase + epinephrine glucogan AMP - G6P insulin ATP HMP shut 1- Glucose 6 phosphate dehydrogenase + NADP -NADPH |
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Rate determining enzymes of metabolic process in De novo pyrimidine synthesis De novo purine synthesis urea cycle |
De novo pyrimidine synthesis 1- carbomoyl phosphate synthase II + ATP, PRPP - UTP De novo purine synthesis 1- Glutamine phosphoribosylpyrophosphate PRPP amidotransferase - AMP IMP GMP Urea cycle 1- Carbomoyl phosphate synthase I +N acetyl glutamate |
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Rate determining enzyme in fatty acid synthesis fatty acid oxidation ketogenesis and cholesterol synthesis |
Fatty acid synthesis 1- Acetyl CoA carboxylase +insulin citrate - Glucogon palmitoyl CoA Fatty acid oxidation 1- Cornitin acyltransferase I - Molonyl CoA Ketogenesis HMG CoA synthase Cholesterol synthesis 1- HMG CoA reductase + insulin thyroxine estrogen - glucogon chelestrol |
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ATP production |
1- Aerobic metabolism of one glucose molecule produces 32 net ATP via malate aspartate shuttle (liver and heart) end product NADH 2- 30 net ATP via glycerol 3 phosphate shuttle (muscle) end produce FADH2 3- Anaerobic glycolysis produced only 2 net ATP per glucose molecule takes place in the cytoplasm 4- ATP hydrolysis can be coupled to energetically unfavorable reaction 5- Arsinic cause glycolysis to produce zero net ATP by inhibiting Pyruvate dehydrogenase |
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A male with garlic breath is noted to have arsenic poisoning from groundwater how many ATP molecules will he produce via glycolysis |
None |
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What supplies the energy to turn substrate into product in energetically unfavorable reactions |
ATP hydrolysis |
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Which organ tissues utilize the malate aspartate shuttle during aerobic metabolism |
Heart Liver |
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Which organ tissue utilizes the glycerol 3 phosphate shuttle pathway during glucose metabolism |
Muscle |
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Activated carriers ATP, NADP/NADPH/FADH, CoA lipomide Biotin Tetrhydrofolate S adenosylmethionin TPP |
ATP - phosphoryl group NADH NADPH FADH - Electrons CoA lipomide - acyl group Biotin - CO group Tetrhydrofolate - 1 Carbon group S adenosylmethionin - CH group TPP- Aldehydes |
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Universal electron acceptors |
1- Nicotinamides (NAD NADP from vitamin B3) 2- Flavin nucleotides (FAD from vitamin B2) 3- NAD is used in catabolic process to carry reducing equivalents away as NADPH 4- NADPH is used in anabolic processes as a supply of reducing equivalent 5- NADPH is a product of the HMP shunt |
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NADPH used in |
Anabolic reaction Cytochrome p450 systems Gluthatione reductase Respiratory burst |
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Anabolic processes generally use which electron acceptor as a supply of reducing equivalent |
NADPH |
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What is the end product of the heroes monophosphate shunt |
NADPH |
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Hekokinase vs glucokinase |
1- Phosphorylation of glucose to glucose 6 phosphate is catalyzed by glucokinase in the liver and beta cells of the pancreas and hexokinase in other tissue 2- Hexokinase sequester glucose in tissue to be used even when glucose concentration is low 3- High levels of glucose glucokinase stores glucose in the liver 4- Glucose 6 phosphate inhibits hexokinase 5- Fructose 6 phosphate inhibits glucokinase 6- Hexokinase have a high affinity and low Vmax 7- Glucokinase have a low affinity and high Vmax induced by insulin |
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Which enzyme has a greater capacity to convert glucose to glucose 6 phosphate glucokinase or hexokinase |
Glucokinase |
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What disease states are associated with Glucokinase deficiency |
Gestational diabetes Maturity onset diabetes of the young |
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What enzyme catalyzes the only reversible reaction in glycolysis that produces ATP |
Phosphoglycerate kinase |
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Which enzyme in glycolysis require ATP |
Glucokinase Fructokinase Phosphofructokinase 1 |
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In glycolysis which two reactions generate ATP |
1,3 Biphosphoglycerate — 3 phosphoglycerate by phosphoglycerate kinase Phosphoenolpyruvate — Pyruvate by Pyruvate kinase |
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In glycolysis the conversion of phosphoenolypyruvate to Pyruvate is irreversibly catalyzed by which enzyme |
Pyruvate kinase |
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What compound inhibit the reaction catalyzed by Phosphofructokinase 1 |
ATP Citrate |
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What compound inhibit the reaction catalyzed by Pyruvate kinase |
ATP Alanine Glucogan |
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Phosphofructokinase-1 catalyzes which step in glycolysis |
Fructose 6 phosphate — fructose 1,6 bisphosphate |
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Name the 2 enzymes that catalyze the conversion of glucose to glucose-6- phosphate |
Glucokinase Hexokinase |
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What enzyme catalyzes the rate limiting step in glycolysis |
Phosphofructokinase-1 |
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What are the net products of glycolysis |
Glucose + 2P + 2ADP + 2NAD — 2Pyruvate + 2ATP + 2NADH + 2 H + 2H2O |
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Where in the cell does glycolysis occur |
Cytoplasm |
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What compound promote the reaction catalyzed by Pyruvate kinase |
Fructose 1,6 bisphosphate |
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What compound promotes the reaction catalyzed by Phosphofructokinase- 1 |
AMP Fructose 2,6 bisphosphate |
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Fructose 2 6 bisphosphate during fasting state |
Increase glucagon Increase cAMP Increase protein kinase A Increase FBPase 2 Decrease PFK Less glycolysis more gluconeogenesis |
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Fructose 2 6 bisphosphate during feeding |
Increase insulin Decrease cAMP Decrease protein kinase A Decrease FBPase2 Increase PFK More glycolysis less gluconeogenesis |
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What enzyme reverse function of fructose 2,6 bisphosphate and Phosphofructokinase 2 |
Pristine Kinase A |
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Which enzyme that regulates the level of fructose 2,6 bisphosphate is active in the fasting state |
Fructose bisphosphatase 2 |
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What reaction is catalyzed by fructose bisphosphatase 2 FBPase 2 |
Fructose 2,6 bisphosphate — fructose 6 phosphate |
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In gluconeogenesis what enzyme catalyzes fructose 1,6 bisphosphate to fructose 6 phosphate |
Fructose bisphosphatase -1 |
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The enzyme Phosphofructokinase 2 catalyzes which reaction |
Fructose 6 phosphate to fructose 2,6 bisphosphate |
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Name the 2 bifunctional enzymes involved in fructose 2,6 bisphophate metabolism |
Fructose bisphosphatase 2 Phospfructokinase 2 |
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What compound promotes the activity of the Phosphofructokinase 1 enzyme |
Fructose 2,6 bisphosphate |
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Pyruvate dehydrogenase complex |
1- Mitochondrial complex linking glycolysis and TCA cycle 2- Differentially regulated in fed(active)/fasting(inactive) state 3- Reaction Pyruvate + CoA + NAD — acytyl CoA + CO2 + NADH 4- Activated by NAD/NADH , ADP, Ca 5- Contains 3 enzymes and 5 cofactors 6- alpha ketoglutarate dehydrogenase complex uses similar cofactors (alpha ketoglutarate to succinyl CoA in TCA cycle ) |
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Pyruvate dehydrogenase complex |
1- Mitochondrial complex linking glycolysis and TCA cycle 2- Differentially regulated in fed(active)/fasting(inactive) state 3- Reaction Pyruvate + CoA + NAD — acytyl CoA + CO2 + NADH 4- Activated by NAD/NADH , ADP, Ca 5- Contains 3 enzymes and 5 cofactors 6- alpha ketoglutarate dehydrogenase complex uses similar cofactors (alpha ketoglutarate to succinyl CoA in TCA cycle ) |
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Cofactors for Pyruvate dehydrogenase complex |
Thiamine pyrophosphate (B1) FAD (B2 riboflavin) NAD (B3 niacin) CoA (B5 panthothenic acid) Lipoic acid |
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In a patient with arsenic poisoning what changes are typically seen on ECG |
Prolonged QT |
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What is the mechanism of arsenic poisoning |
Inhibit lipoic acid (cofactor for Pyruvate dehydrogenase) wh |
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Where is the Pyruvate dehydrogenase complex found in the cell |
Mitochondria |
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What are the clinical finding of arsenic poisoning |
Skin pigment changes Skin cancer Vomiting Diarrhea QT prolongation |
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Pyruvate dehydrogenase complex deficiency |
1- X linked 2- Causes a buildup of Pyruvate that is shunted to lactate (LDH) and alanine (ALT) 3- Finding neurological defects lactic acidosis and increase serum alanine 4- Treatment- increase intake of ketogenic nutrients (high fat diet increase lysine and leucine) |
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Findings of Pyruvate dehydrogenase complex deficiency |
Neurological defects Lactic acidosis Increase alanine |
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Treatment of Pyruvate dehydrogenase complex deficiency |
Increase intake of ketogenic nutrients High fat diet Increase leucine and lysine |
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Pyruvate metabolism |
Alanine aminotransferase B6 1- alanine carries amino group to the liver from the muscle Pyruvate carboxylase (biotin) 1- oxaloacetate can replenish TCA cycle or be used in gluconeogenesis Pyruvate dehydrogenase B1 B2 B3 B5 lipoic acid 1- Transition from glycolysis to TCA cycle Lactic acid dehydrogenase 1- End of anaerobic glycolysis |
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Location of anaerobic glycolysis |
RBC WBC Lens Cornea Renal medulla Testes |
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Where in the cell do Pyruvate dehydrogenase and Pyruvate carboxylase act |
Mitochondria |
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Where in the cell do Pyruvate dehydrogenase and Pyruvate carboxylase act |
Mitochondria |
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Where in the cell does alanine aminotransferase and lactic acid dehydrogenase act |
Cytoplasm |
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During Pyruvate metabolism which enzyme produces CO2 as by product |
Pyruvate dehydrogenase |
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Pyruvate is converted into which 2 molecules that enter the TCA cycle |
Acetyl CoA Oxaloacetate |
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Of the 4 Pyruvate metabolic pathway which requires ATP and carbon dioxide |
Pyruvate carboxylase |
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Which amino acid carriers amino groups from muscle to liver |
Alanine |
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What products are produce from each Pyruvate metabolism |
Alanine aminotransferase- alanine Pyruvate carboxylase- Oxaloacetate Pyruvate dehydrogenase- Acetyl CoA Laxative acid dehydrogenase- lactate |
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A patient has vitamin B3 deficiency what enzymes of the Pyruvate metabolism will be affected |
Pyruvate dehydrogenase Lactic acid dehydrogenase |
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Of the 4 Pyruvate metabolic pathway which requires NADH |
Lactic acid dehydrogenase |
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Of the 4 Pyruvate metabolic pathway which require NAD |
Pyruvate dehydrogenase |
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What are the associated cofactors of alanine aminotransferase Pyruvate carboxylase lactic acid dehydrogenase |
B6 Biotin B3 |
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TCA cycle function |
1- Also know as Krebs cycle 2- Glycolysis produce 1 NADH and 1 CO2 3- TCA produces 3NADH 2CO 1 FADH 1GTP total of 10ATP/acetyl CoA x2 if per glucose 4- Occurs in mitochondria 5- alpha ketoglutarate uses the same cofactors as Pyruvate dehydrogenase |
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Irreversibly enzymes of the Krebs cycle |
Pyruvate dehydrogenase Citrate syntheses Isocitrate dehydrogenase Alpha ketoglutarate dehydrogenase |
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Kreb cycle flow diagram |
Back (Definition) |
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What is the order of substrates of the tricarboxylic acid (TCA) cycle after acetyl CoA enters |
Citrate Isocitrate Alpha ketoglutarate Succinyl CoA Succinate Fumerate Malate Oxaloacetate |
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What intermediate of TCA cycle provides negative feedback to alpha ketoglutarate dehydrogenase to inhibit its function |
Succinyl CoA |
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Cofactors required for alpha ketoglutarate dehydrogenase complex |
B1 B2 B3 B5 Lipoic acid |
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A which steps in the tricarboxylic cycle is NADH produce |
Isocitrate - alpha ketoglutarate Alpha ketoglutarate- succinyl CoA Malate - oxaloacetate |
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In the tricarboxylic acid cycle ATP inhibits which 3 enzymes |
Citrate synthase Isocitrate dehydrogenase Alpha ketoglutarate dehydrogenase |
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In the tricarboxylic acid cycle which steps produce CO2 |
Isocitrate- alpha ketoglutarate Alpha ketoglutarate- succinyl CoA |
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How many ATP molecules are produced during 1 turn of the TCA cycle |
10 ATP for each acetyl CoA |
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In the TCA cycle which compounds inhibit Isocitrate dehydrogenase |
ATP NADH |
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How many NADH molecules are produced during 1 turn of the TCA cycle |
3 |
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How many FADH molecules are produced during 1 turn of TCA cycle |
1 |
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How many CO2 molecules are produced during 1 turn of TCA cycle |
2 |
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In the TCA Cycle which compound activates Isocitrate dehydrogenase |
ADP |
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How many ATP molecules are produced from 1 glucose molecule in the TCA |
20 |
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A which step in the TCA cycle is GTP produce |
Succinyl CoA- Succinate |
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A which steps in the TCA cycle is FADH produce |
Succinate - fumerate |
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What 3 compounds inhibit the activity of Pyruvate dehydrogenase |
Acetyl CoA NADH ATP |
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How much GTP is produced in the TCA cycle |
1 |
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What are the products of the enzymatic activity of Pyruvate dehydrogenase on Pyruvate |
2 acetyl CoA CO2 NADH |
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Electron transport chain and oxidative phosphorylation |
1- NADH electrons from glycolysis enter the notice via malate aspartate and glycerol 3 phosphate shuttle 2- FADH electrons transferred to complex 2 (lower eagerly level from NADH) 3- The passage of electrons leads to the formation of proton gradients 4- Proton gradient coupled oxidative phosphorylation to drive production of ATP 5- NADH 2.5 ATP FADH 1.5 ATP |
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Electron transport chain and oxidative phosphorylation |
1- NADH electrons from glycolysis enter the notice via malate aspartate and glycerol 3 phosphate shuttle 2- FADH electrons transferred to complex 2 (lower eagerly level from NADH) 3- The passage of electrons leads to the formation of proton gradients 4- Proton gradient coupled oxidative phosphorylation to drive production of ATP 5- NADH 2.5 ATP FADH 1.5 ATP |
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Electron transport inhibitors |
1- Directly inhibits electron transport 2- Decrease proton gradient and block ATP synthase 3- Rotenone - complex 1 inhibitor Actinomycin complex 3 inhibitor Cyanide carbon monoxide azide complex 4 inhibitor |
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Electron transport chain and oxidative phosphorylation |
1- NADH electrons from glycolysis enter the notice via malate aspartate and glycerol 3 phosphate shuttle 2- FADH electrons transferred to complex 2 (lower eagerly level from NADH) 3- The passage of electrons leads to the formation of proton gradients 4- Proton gradient coupled oxidative phosphorylation to drive production of ATP 5- NADH 2.5 ATP FADH 1.5 ATP |
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Electron transport inhibitors |
1- Directly inhibits electron transport 2- Decrease proton gradient and block ATP synthase 3- Rotenone - complex 1 inhibitor Actinomycin complex 3 inhibitor Cyanide carbon monoxide azide complex 4 inhibitor |
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ATP synthase inhibitor |
1- Directly inhibit ATP Synthase 2- Increase proton gradient 3- No ATP produce because electron transport stop 4- Oligomycin |
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Electron transport chain and oxidative phosphorylation |
1- NADH electrons from glycolysis enter the notice via malate aspartate and glycerol 3 phosphate shuttle 2- FADH electrons transferred to complex 2 (lower eagerly level from NADH) 3- The passage of electrons leads to the formation of proton gradients 4- Proton gradient coupled oxidative phosphorylation to drive production of ATP 5- NADH 2.5 ATP FADH 1.5 ATP |
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Electron transport inhibitors |
1- Directly inhibits electron transport 2- Decrease proton gradient and block ATP synthase 3- Rotenone - complex 1 inhibitor Actinomycin complex 3 inhibitor Cyanide carbon monoxide azide complex 4 inhibitor |
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ATP synthase inhibitor |
1- Directly inhibit ATP Synthase 2- Increase proton gradient 3- No ATP produce because electron transport stop 4- Oligomycin |
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Uncoupling agents |
1- Increase permeability of the membrane 2- Decrease proton gradient 3- Increase oxygen consumption 4- No ATP produce but electron transport continue 5- Produce heat 6- 2,4 dimitrophenol (used illicitly for weight loss) Aspirin( cause fever after overdose) Thermogenin in brown fat (has more mitochondria than white fat) |
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Electron transport chain and oxidative phosphorylation |
1- NADH electrons from glycolysis enter the notice via malate aspartate and glycerol 3 phosphate shuttle 2- FADH electrons transferred to complex 2 (lower eagerly level from NADH) 3- The passage of electrons leads to the formation of proton gradients 4- Proton gradient coupled oxidative phosphorylation to drive production of ATP 5- NADH 2.5 ATP FADH 1.5 ATP |
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Electron transport inhibitors |
1- Directly inhibits electron transport 2- Decrease proton gradient and block ATP synthase 3- Rotenone - complex 1 inhibitor Actinomycin complex 3 inhibitor Cyanide carbon monoxide azide complex 4 inhibitor |
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ATP synthase inhibitor |
1- Directly inhibit ATP Synthase 2- Increase proton gradient 3- No ATP produce because electron transport stop 4- Oligomycin |
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Uncoupling agents |
1- Increase permeability of the membrane 2- Decrease proton gradient 3- Increase oxygen consumption 4- No ATP produce but electron transport continue 5- Produce heat 6- 2,4 dimitrophenol (used illicitly for weight loss) Aspirin( cause fever after overdose) Thermogenin in brown fat (has more mitochondria than white fat) |
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Name of complexes in electron transport chain |
Complex 1 Complex 2- Succinate dehydrogenase Complex 3- Complex 4- Cytochrome C oxidase Complex 5- ATP synthase |
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How does the passage of electrons from NADH and FADH produce ATP |
Results in a proton gradient through which oxidative phosphorylation produces ATP |
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Why does FADH produce fewer molecules of ATP than NADH |
At a lower energy level Complex 2 |
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What conpound directly inhibits complex 1 |
Rotenone |
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What compond directly inhibits complex 3 |
Actinomycin |
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What compound directly inhibits complex 4 |
Cyanide Carbon monoxide Asides |
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What role does thermogenin play in white and brown fat |
Thermogenin in brown fat have more mitochondria and produces more heat than white fat |
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List the 6 most common inhibitors of the electron transport chain |
2, 4 Dinitrophenol Rotenone Actinomycin A Cyanide CO Oligomycin |
DRACCO |
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What enzyme produces ATP as the end product of oxidative phosphorylation |
ATP synthase |
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Pyruvate carboxylase in gluconeogenesis |
1-In mitochondria 2-Pyruvate to oxaloacetate 3-Require biotin and ATP 4-Activated by acetyl CoA |
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Phosphoenolpyruvate carboxylase |
1- In cytosol 2- Oxaloacetate to phosphoenolpyruvate 3- Require GTP |
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Phosphoenolpyruvate carboxylase |
1- In cytosol 2- Oxaloacetate to phosphoenolpyruvate 3- Require GTP |
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Fructose 1,6 bisphosphatase in gluconeogenesis |
1- In cytosol 2- Fructose 1,6 bisphosphate to fructose 6 phosphate 3- + citrate - AMP - fructose 2,6 bisphosphate |
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Glucose 6 phsphatase in gluconeogenesis |
1- In ER 2- Glucose 6 phosphate to glucose |
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Gluconeogenesis irreversibly enzymes |
1- Occurs in the liver, maintain euglycemia during fasting 2- Enzyme also found in the kidney and intestinal epithelium 3- Deficiency in key gluconeogenesic enzyme cause hypoglycemia 4- Muscle cannot participate gluconeogenesis because it lacks glucose 6 phosphate 5- Odd chain fatty acid yield 1 propionyl CoA during metabolism that enters the TCA cycle as succinyl CoA undergoes gluconeogenesis and serve as a glucose source 6- Even chain fatty acid cannot produce glucose because it only yield acetyl CoA equivalent |
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4 gluconeogenesis irreversibly enzymes |
Pyruvate carboxylase Phosphoenolpyruvate Fructose 1,6 bisphosphatase Glycerol 6 phosphatase |
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What are the inhibitors of fructose 1,6 bisphosphate |
AMP Fructose 2,6 bisphosphate |
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Why are even chain fatty acids unable to produce new glucose |
Yield acetyl CoA equivalent |
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Pentose phosphate pathway |
1- Also called HMP (hexose monophosphate )shunt 2- Provide a source of NADPH from abundantly available glucose 6 phosphate 3- NADPH is required for reductive reaction eg glucothioine in RBC fatty acid and cholesterol 4- Yield ribose for nucleotide synthesis 5- Two phases oxidative and non oxidative both takes place in the cytoplasm 6- No ATP is used or produced 7- Location lactating mammary glands, liver, adrenal cortex (steroid synthesis) and RBC |
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Oxidative phase of HMP Shunt |
Glucose 6 phosphate to 6 phophogluconate by glucose 6 P dehydrogenase produce NADPH 6 phosphogluconate to ribulose 5 P produce NADPH and CO2 |
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Non oxidative/reversible phase of HMP shunt |
Glucose 6 P to Fructose 6 P to fructose 1,6 phosphate to DHAP and glyceroaldehde 3 phosphate Ribulose 5 P to ribose 5 p by phosphopentose isomerase Ribose 5 phosphate to fructose 6 P and glyceraldehyde by transketolase B1 |
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In oxidative stress where will the pentose phosphate pathway be up regulated in response |
RBC |
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What is the function of NADPH in RBC |
Reduces glutathione |
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What is the rate limiting enzyme in HMP shunt |
Glucose 6 phosphate dehydrogenase |
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What molecule acts as an inhibitor of the oxidative phase of HMP shunt |
NADPH |
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Pentose phosphate pathway source of which 2 molecules required for cellular processes |
NADPH Ribose |
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What are the 3 products of the non oxidative phases of pentose phosphate pathway |
Ribose 5 phosphate Fructose 6 phosphate Glyceraldehyde 3 phosphate |
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Glucose 6 phosphate dehydrogenase deficiency demographics |
1- X linked recessive disorder 2- Most common inherited enzyme deficiency 3- More prevalent in blacks 4- Increase Malarial resistance 5- Heinz bodies- denatured globulin chains precipitated within RBC due to oxidative stress 6- Bite cells - phagocytic removal of Heinz body by splenic macrophages |
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Glucose 6 phosphate dehydrogenase deficiency demographics |
1- X linked recessive disorder 2- Most common inherited enzyme deficiency 3- More prevalent in blacks 4- Increase Malarial resistance 5- Heinz bodies- denatured globulin chains precipitated within RBC due to oxidative stress 6- Bite cells - phagocytic removal of Heinz body by splenic macrophages |
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Oxidative agents in G6PD deficiency |
Fava beans Sulphonamide Nitrofurantoin Primaquine/chloroquine Anti TB drugs
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Glucose 6 phosphate dehydrogenase deficiency demographics |
1- X linked recessive disorder 2- Most common inherited enzyme deficiency 3- More prevalent in blacks 4- Increase Malarial resistance 5- Heinz bodies- denatured globulin chains precipitated within RBC due to oxidative stress 6- Bite cells - phagocytic removal of Heinz body by splenic macrophages |
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Oxidative agents in G6PD deficiency |
Fava beans Sulphonamide Nitrofurantoin Primaquine/chloroquine Anti TB drugs
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Glucose 6 phosphate dehydrogenase deficiency |
1- NADPH is necessary to keep glutathione reduce 2- It detoxifies free radicals and peroxide’s 3- Decrease NADPH in RBC causes hemolytic anemia due to poor RBC defense against oxidative agents 4- Infection most common precipitant for hemolysis 5- Inflammatory response produces free radicals that diffuse in RBC causing oxidative damage |
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What enzyme catalyzes the conversion of peroxide to water |
Glutathione peroxidase |
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Glucose 6 phosphate dehydrogenase changes glucose 6 phosphate into which substrate |
6 Phosphoglucolactone |
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Which substrate detoxifies free radicals and peroxide’s in the cell |
Glutathione |
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The enzyme glutathione reductase uses which substrate to reduce glutathione |
NADPH |
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Essential Fructosuria |
1- Autosomal recessive 2- Defect in fructokinase 3- Benign and asymptomatic because fructose is not trap in the cell 4- Hexokinase become the primary pathway for converting fructose to fructose 6 phosphate 5- Symptoms fructose in blood and urine 6- Milder symptoms than analogous disorders of galactose |
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Essential Fructosuria |
1- Autosomal recessive 2- Defect in fructokinase 3- Benign and asymptomatic because fructose is not trap in the cell 4- Hexokinase become the primary pathway for converting fructose to fructose 6 phosphate 5- Symptoms fructose in blood and urine 6- Milder symptoms than analogous disorders of galactose |
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Hereditary fructose intolerance |
1- Autosomal recessive 2- Hereditary deficiency of Aldolase B 3- Fructose 1 phosphate accumulates which decrease availability of phosphate 4- Results in inhibition of Glycogenlysis and gluconeogenesis 5- Symptoms 1- negative urine dipstick (only test for glucose) 2- reducing sugar in urine 3- Presents following consumption of fruits juice and honey 4 hypoglycemia jaundice cirrhosis vomiting 6- Treatment- decrease intake of fructose, sucrose and sorbitol |
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Is essential fructosuria or fructose intolerance more clinically significant |
Fructose intolerance because fructose 1 phosphate increase decreasing availability of phosphate for ATP which is needed for Glycogenlysis and gluconeogenesis |
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What intermediate in fructose metabolism directly feeds into glycolysis |
Glyceraldehyde 3 phosphate |
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What enzyme converts dihydroxyacetone phosphate to glyceraldehyde 3 phosphate |
Triose phosphate isomerase |
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What enzyme converts glyceraldehyde to glyceraldehyde 3 phosphate |
Triose kinase |
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Galactokinase deficiency |
1- Autosomal recessive 2- Defect in galacotokinase 3- Gactotitol accumulates when galactose is present in the diet 4- Mild condition 5- Symptoms galactose in blood galactosemia Galactose in urine galactouria Infantile cat act 6- May present as failure to track objects or to develop a social smile |
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Galactokinase deficiency |
1- Autosomal recessive 2- Defect in galacotokinase 3- Gactotitol accumulates when galactose is present in the diet 4- Mild condition 5- Symptoms galactose in blood galactosemia Galactose in urine galactouria Infantile cat act 6- May present as failure to track objects or to develop a social smile |
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Classic galactosemia |
1- Autosmal recessive 2- Hereditary deficiency of galactose 1 phosphate uridyltransferase 3- Damage due to the accumulation of toxic substances 4- Galactitol accumulating in lens of the eye 5- Symptoms 1- Develop at the beginning of infantile feeding( lactose present in breast milk and formula) 2- Infantile cataract, jaundice, hepatomegaly, failure to thrive, intellectual disability 3- Increase risk of E. coli sepsis in infants 6- Treatment - avoid galactose and lactose in diet |
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What enzyme catalyzes production of galctitol |
Aldose reductase |
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What 2 milestone abnormalities might be present in a toddler with recently diagnosed galactokinase deficiency |
Failure to track object and decay social smile |
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What cofactor is required for conversion of galactose 1 phosphate to glucose 1 phosphate |
UDP glucose |
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Severe galactose 1 phosphate uridyltransferase deficiency results in depletion of which molecule |
Phosphate |
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Symptoms of classic galactosemia |
1- Develops at the beginning of feeding for infants (lactose in breast mile and formula 2-Infantile cataract Jaundice Hepatomegaly Failure to thrive Intellectual disability 3- Increase risk of E. coli sepsis in infants |
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In galactose metabolism a block in the conversion of galactose to galactose 1 phosphate is caused by what disease |
Galactokinase deficiency |
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In galactose metabolism galactose 1 phosphate is converted to glucose 1 phosphate by which enzyme |
Galactose 1 phosphate uridyltransferase |
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Which enzyme regenerates UDP glucose from UDP galactose |
4 epimerase |
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Sorbitol |
1- Alternative methods of trapping glucose in the cell is by converting it to its alcohol counterpart sorbitol via aldose reductase 2- Some tissue the convert sorbitol to fructose via sorbitol dehydrogenase 3- Tissues with insufficiency amount/activity of sorbitol dehydrogenase cause increase risk of intracellular sorbitol accumulation causing osmotic damage (cataract, retinopathy and peripheral neuropathy seen with chronic hyperglycemia in diabetics) 4- High blood levels of galactose can be converted to its osmotically active galactitol via aldose reductase 5- Liver, Ovaries and Seminal vesicles have both enzymes 6- Lens have primarily aldose reductase 7- Retinal kidney and Schwann cells have aldose reductase |
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Beside glucose what other sugar is also converted to its respective osmotically active alcohol form via aldose reductase |
Galactose to Galactitol |
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What cofactor is used by aldose reductase |
NADPH |
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How is sorbitol cleared from cells in some tissue |
Convert sorbitol to fructose via sorbitol dehydrogenase |
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What is the cofactor used by the enzyme sorbitol dehydrogenase |
NAD |
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What 3 tissues have both aldose reductase and sorbitol dehydrogenase |
Liver Ovaries Seminal vesicles |
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Which enzyme is primarily involved in sorbitol metabolism in the lens |
Aldose reductase |
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Cells in what 3 tissues have only aldose reductase |
Retina Kidney Schwann cells |
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Lactate deficiency |
1- Insufficient lactate enzyme - Dietary lactose deficiency 2- Lactate function on the intestinal brush boarder to digest lactose into glucose and galactose 3- Primary - age dependent decrease after childhood absent lactose persistent alle Seen in Asians Africans and Native American descend 4- Secondary loss of intestinal brush boarder to gastroenteritis autoimmune disease 5- Congenital lactose deficiency- rare digestive gene 6- Stool demonstrate decrease pH and breast test increase hydrogen content with lactose hydrogen breath test 7- Intestinal biopsy shows normal mucosa with hereditary lactose intolerance |
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Lactate deficiency |
1- Insufficient lactate enzyme - Dietary lactose deficiency 2- Lactate function on the intestinal brush boarder to digest lactose into glucose and galactose 3- Primary - age dependent decrease after childhood absent lactose persistent alle Seen in Asians Africans and Native American descend 4- Secondary loss of intestinal brush boarder to gastroenteritis autoimmune disease 5- Congenital lactose deficiency- rare digestive gene 6- Stool demonstrate decrease pH and breast test increase hydrogen content with lactose hydrogen breath test 7- Intestinal biopsy shows normal mucosa with hereditary lactose intolerance |
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Findings of lactase deficiency |
Bloating Cramping Osmotic diarrhea Flatulence |
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Lactate deficiency |
1- Insufficient lactate enzyme - Dietary lactose deficiency 2- Lactate function on the intestinal brush boarder to digest lactose into glucose and galactose 3- Primary - age dependent decrease after childhood absent lactose persistent alle Seen in Asians Africans and Native American descend 4- Secondary loss of intestinal brush boarder to gastroenteritis autoimmune disease 5- Congenital lactose deficiency- rare digestive gene 6- Stool demonstrate decrease pH and breast test increase hydrogen content with lactose hydrogen breath test 7- Intestinal biopsy shows normal mucosa with hereditary lactose intolerance |
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Findings of lactase deficiency |
Bloating Cramping Osmotic diarrhea Flatulence |
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Treatment for lactase deficiency |
Avoid dairy products Add Lactase pill to diet lactose free milk |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Glucogenic amino acid |
Methionine Histidine Valine |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Glucogenic amino acid |
Methionine Histidine Valine |
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Glucogenic/ketogenic amino acids |
Phenylalanine Tryptophan Threanine Isoleucine |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Glucogenic amino acid |
Methionine Histidine Valine |
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Glucogenic/ketogenic amino acids |
Phenylalanine Tryptophan Threanine Isoleucine |
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Ketogenic amino acid |
Leucine Lysine |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Glucogenic amino acid |
Methionine Histidine Valine |
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Glucogenic/ketogenic amino acids |
Phenylalanine Tryptophan Threanine Isoleucine |
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Ketogenic amino acid |
Leucine Lysine |
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Acidic amino acid |
Aspartic acid Glutamic acid Negatively charged at body pH |
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Essential amino acid |
Phenylalanine Valine Tryptophan Threanine Isoleucine Methionine Histidine Leucine Lysine |
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Glucogenic amino acid |
Methionine Histidine Valine |
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Glucogenic/ketogenic amino acids |
Phenylalanine Tryptophan Threanine Isoleucine |
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Ketogenic amino acid |
Leucine Lysine |
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Acidic amino acid |
Aspartic acid Glutamic acid Negatively charged at body pH |
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Basic amino acid |
1- Histidine, Lysin and Arginine 2- Arginine most basic 3- Histidine have no charge at body pH 4- Arginine and histidine are required during periods of growth 5- Arginine and Lysin are increased in histones which bind negatively charged DNA |
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Which amino acid isomer is found in the human body |
L amino acids |
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Urea cycle |
1- Amino acid catabolism is required for the formation of common metabolites 2- Source of metabolic fuel 3- Excess nitrogen generated during this process is converted to urea and excreted by the kidney 4- Occurs in the liver mitochondria and cytoplasm 5- Require 3 ATP |
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Mitochondria metabolic site |
1- fatty acid oxidation (beta oxidation) 2- Acetyl CoA production 3- TCA cycle 4- Ketogenesis 5- Oxadative phosphorylation |
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Glycolysis regulation key enzymes |
1- Occur in cytoplasms 2- Glucose + 2P + 2ADP + 2NAD = 2 Pyruvate + 2ATP + 2NADH + 2H + 2H2O 3- Equation not balanced chemically and exact balance equation depends on ionization state of reactants and products 4- Required ATP glucose to glucose 6 phosphate via Hexokinase/Glucokinase Fructose to fructose 6 phosphate via Phosphofructokinase 5- Produce ATP 1,3 BPG to 3PG via phosphoglycerate kinase Phosphoenolpyruvate to Pyruvate via Pyruvate kinase + fructose 1,6 bisphosphate - ATP Alanine |
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