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74 Cards in this Set
- Front
- Back
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How many ATP are produced in aerobic metabolism?
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32 net ATP via malate-aspartate shuttle
30 net ATP via glycerol-3-3phosphate shuttle |
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How many ATP are produced in anaerobic glycolysis?
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2 net ATP per glucose molecule
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What are the location, Km(affinity), and Vmax(capacity) of Hexokinase?
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-Location: most tissues, but not liver nor B cells of pancreas
Km: low glucose concentrations increases affinity Vmax: low glucose concentrations decreases capacity |
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What are the location, Km(affinity), and Vmax(capacity) of Glucokinase ?
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Location: liver, B cells of pancreas
Km: High glucose concentrations decreases affinity Vmax: High glucose concentrations increases capacity |
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What are the function of insulin ?
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-Increases glucose transport in skeletal muscle and adipose tissue, glycogen synthesis and store, triglyceride synthesis, NA+ retention, protein synthesis, and cellular uptake of K+ and amino acids
-Decrease glucagon release |
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What are the insulin-independent glucose transporters?
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GLUT-1: RBCs, brain, cornera
GLUT-2: B islet cells, liver, kidney, small intestine GLUT-5: Spermatocytes, GI tract |
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What are the insulin-dependent glucose transporters?
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GLUT-4: Adipose tissue, skeletal muscle
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What are the most important enzymes in glycolysis?
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-Hexokinase/glucokinase
-Phosphofructokinase-1 -Pyruvate kinase |
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What are the most important enzymes in gluconeogenesis ?
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-Glucose-6-phosphatase
-Fructose-1,6-bisphophatase -Pyruvate carboxylase -PEP carboxykinase |
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Why can't muscles participate in gluconeogenesis ? |
It lacks glucose-6-phosphatase |
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What are the effects of Rotenone, cyanide, antimycin A, CO (electron transport inhibitors) |
Directly inhibit electron transport, causing a decreased proton gradient and block of ATP synthesis |
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What are the effects of Uncoupling agents like 2-4-dinitrophenol and aspirin ?
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Increases permeability of membrane, causing a decreases in proton gradient and increases in O2 consumption. ATP synthesis stop
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What is the rate-determining enzyme of glycolysis ?
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Phosphofructokinase-1
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What is the rate-determining enzyme of gluconeogenesis ?
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fructose-1-6-bisphosphatase
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What is the rate-determining enzyme of TCA cylce ?
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Isocitrate dehydrogenase
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What is the rate-determining enzyme of glycogenesis ?
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Glycogen synthase
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What is the rate-determining enzyme of glycogenolysis ?
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Glycogen phosphorylase
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What is the rate-determining enzyme of HMP shunt ?
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Glucose-6-phosphate dehydrogenase
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What is the rate-determining enzyme of De novo pyrimidine synthesis ?
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Carbamoyl phosphate dehydrogenase
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What is the rate-determining enzyme of De novo purine synthesis ?
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Glutamine-phosphoribosylpyrophosphate (PRPP) amidotransferase
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What is the rate-determining enzyme of urea cycle ?
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Carbamoyl phosphate synthese 1
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What is the rate-determining enzyme of fatty acid synthesis ?
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Acetyl-CoA carboxylase
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What is the rate-determining enzyme of fatty acid oxidation ?
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Carnitine acyltransferase 1
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What is the rate-determining enzyme of ketogenesis? |
HMG-CoA synthesis |
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What is the rate-determining enzyme of cholesterol synthesis? |
HMG-CoA reductase |
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What disease is produced by a Glucose-6-phosphatase deficiency? |
Von Gierke disease (type 1) |
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Autosomal recessive disease that produces a severe fasting hypoglycemia
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Von Gierke disease (type 1)
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What is the treatment of Von Gierke disease (type 1) ?
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-Oral glucose
-Avoidance of fructose and galactose |
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What disease is produced by a Lysosomal alfa-1,4-glucosidase deficiency ?
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Pompe disease (type 2)
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What disease is produced by a Debraching enzyme alfa-1,6-glucosidase deficiency? |
Cori disease (type 3) |
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What disease is produced by a Skeletal muscle glycogen phosphorylase deficiency ?
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Mcardle disease (type 4)
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Autosomal recessive disease that blocked degradation of branched amino acids (isoleucine, leucine, valine) due to a decreases in alfa-ketoacid dehydrogenase
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Maple syrup urine disease
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What are the most important enzymes in TCA cycle ?
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-Citrate synthase
-Isocitrate dehydrogenase -Alfa-KG dehydrogenase |
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Disease that can be acquired or herediraty, results in excess of NH4+, which depletes alfa-ketoglutarate, leading to inhibition of TCA cycle
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Hyperammonemia
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What are the SE of Ammonia intoxication ?
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-Tremor
-Slurring of speech -Somnolence -Vomiting -Cerebral edema -Blurring of vision |
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What is the treatment of Hyperammonemia ?
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-Limit protein in diet
-Benzoate or Phenylbutyrate -Lactulose |
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Most common urea cycle disorder
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Ornithine transcarbamylase deficiency
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X-linked disorder that interferes with the body's ability to eliminate ammonia and ornitine |
Ornithine transcarbamylase deficiency |
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Pathway that provides a source of NADPH from abundantly available glucose-6-P, additionally this pathway yields ribose for nucleotide synthesis and glycolytic intermediates
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HMP SHUNT pathway
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X-linked recessive disorder, most common human enzyme deficiency
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Glucose-6-phosphate dehydrogenase deficiency
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Oxidized hemoglobin precipitated within RBCs
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Heinz Bodies
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Result from the phagocytic removal of heinz bodies by splenic macrophages
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Bites cells
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Autosomal recessive disorder that involve a defect in fructokinase, symptoms includes fructose appears in blood and urine
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Essential fructosuria
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Hereditary deficiency of aldolase B, which results in inhibition of glycogenolysis and gluconeogenesis |
Fructose intolerance |
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Hereditary deficiency of galactokinase, symptoms includes galactose appears in blood and urine, infantile cataracts
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Galactokinase deficiency
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Absence of galactose-1-phosphate uridyltransferase |
Classic galactosemia |
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What enzyme convert glucose to sorbitol ?
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Aldose reductase
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What enzyme convert sorbitol to fructose ?
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Sorbitol dehydrogenase
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What is the clinical presentation of lactase deficiency ?
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-Bloating
-Cramps -Flatulence -Osmotic diarrhea |
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Inability to transport LCFAs into the mitochondria, resulting in toxic accumulation, cause weakness, hypotonia, and hypoketotic hypoglycemia
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Carnitine deficiency
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Deficiency that increases dicarboxylic acids and decreases glucose and ketones, and acetyl-COA |
Acyl-CoA dehydrogenase deficiency |
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What is the rate-limiting enzyme of fatty acid synthesis ?
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Acetyl-CoA carboxylase
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What is the rate-limiting enzyme of fatty acid degradation ?
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Carnitine Acyltransferase-1
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What is the rate-limiting enzyme of Ketone bodies ?
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HMG-CoA synthase
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What metabolic scenario favors the synthesis of ketone bodies ?
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When production of acetyl-coa from beta oxidation of fatty acid exceeds the oxidative capacity of the TCA
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Protein malnutrition that result in skin lesions, edema, liver malfunction, and anemia
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Kwashiorkor
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Total calorie malnutrition that result in tissue and muscle wasting, loss of subcutaneous fat, and variable edema
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Marasmus
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What enzyme convert ethanol to acetaldehyde?
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Alcohol dehydrogenase
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What enzyme convert acetaldehyde to acetate ?
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acetaldehyde dehydrogenase
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What drug inhibit acetaldehyde dehydrogenase ?
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Disulfiram
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What drug inhibit alcohol dehydrogenase ?
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Fomepizole
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LDL transports cholesterol from____to_____
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From liver to tissues
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HDL transports cholesterol from________to_____
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From periphery to liver
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What enzyme is deficient in Hyper-chylomicronemia ?
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Lipoprotein lipase or altered apolipoprotein C-II
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Autosomal dominat disease that have absent or defective LDL receptors, cause accelerated atherosclerosis, xanthomas, and corneal arcus
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II-a-familial hyper-cholesterolemia
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Autosomal dominta disease that have a hepatic overproduction of VLDL
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IV-hyper-triglyceridemia
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Autosomal recessive disease, present in childhood with failure to thrive, steatorrhea, acanthocytosis,ataxia, and night blindness that decrease synthesis of apolipoprotein B
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Abetalipoproteinemia
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What is the treatment of abetalipoproteinemia ?
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Vitamin E
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What is the rate-limiting enzyme of cholesterol synthesis ?
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HMG-CoA reductase
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Plaques or nodules composed of lipid-laden histiocytes in the skin, especially the eyelids
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Xanthomas
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Lipid deposit in tendon, especially achilles tendon
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Tendinous xanthoma
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Lipid deposit in cornea, appears early in life with hypercholesterolemia, common in elderly
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Corneal arcus
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Uncommon disease that produced calcification in the media of the arteries, especially radial or ulnar
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Monckeberg (madial calcific sclerosis )
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What are the tow type of Arteriolosclerosis ?
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-Hyaline
-Hyperplastic |
