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173 Cards in this Set
- Front
- Back
What is the most concentrated storage form of fuel? |
Fats (mostly hydrocarbon)! |
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Fatty acids are broken down into what? |
Acetyl CoA |
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Fatty acids are stored as what? |
Triacylglycerols |
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Fatty acids are degraded and synthesized in what number units? |
2-carbon units! |
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TRUE OR FALSE: The key intermediates between fatty acid degradation and synthesis are not the same. |
FALSE. They are the same. |
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What is perilipin? |
Protein that once phosphorylated by Protein Kinase A makes triacylglycerols more accessible to mobilization and triggers the release of the coactivator adipose triglyceride lipase. |
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Triacylglycerols in adipose tissues are converted into fatty acids in response to what? |
Hormonal signals that activate the 7TM receptors that activate cAMP and Protein Kinase A. |
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In hormonal regulation of Lipase, protein kinase A activates triacylglycerol lipase which does what? |
It releases a fatty acid from triacylglycerol converting it into diacylglycerol |
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Glycerol released from fat by lipase is sent to what? |
Glycolysis pathway! |
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What completes the mobilization of fatty acids with the production of a free fatty acid and glycerol? |
Monoacylglycerol lipase |
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What 2 things induce lipolysis? |
1. Epinephrine 2. Glucagon |
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What is albumin? |
Blood protein that serves as a carrier for insoluble fatty acids in the blood stream. |
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What are the 3 steps to convert glycerol into glyceraldehyde 3-phosphate? When is NADH produced? |
1. Glycerol to L-Glycerol 3-phosphate 2. L-Glycerol 3-phosphate to Dihydroxyacetone phosphate (NADH IS PRODUCED) 3. Dihydroxyacetone phosphate to glyceraldehyde 3-phosphate |
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Once converted to glycerol, what two pathways can glycerol undergo? |
1. Glycolysis to Pyruvate 2. Gluconeogenesis to Glucose |
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Where are fatty acids oxidized? |
Mitochondrial matrix |
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In order to be oxidized, fatty acids must be activated by what? |
CoA! |
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What are the 2 steps to fatty acid activation? |
1. Fatty acid reacts with ATP to form acyl adenylate and pyrophosphate
2. Sulfhydryl group of CoA attacks the acyl adenylate to form acyl CoA and AMP |
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How do activated fatty acids cross the inner mitochondrial membrane? |
They must be linked to the alcohol carnitine |
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What are the 4 basic reactions of fatty acid degradation (The Beta Oxidation Pathway)? |
1. Oxidation to form alphabeta double bond (FADH2) 2. Hydration across the double bond 3. Oxidation (NADH) 4. Thiolysis by CoA to displace the first 2 carbons |
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What is the first step of fatty acid breakdown? |
Acyl CoA is oxidized via FAD (forming FADH2) to form trans-delta-enoyl CoA |
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What is the second step of fatty acid breakdown? |
Trans-delta-enoyl CoA is hydrated to form L-3-hydroxyacyl CoA |
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What is the third step of fatty acid breakdown? |
L-3-hydroxyacyl CoA is oxidized via NAD+ (forming NADH) to form 3-ketoacyl CoA |
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What is the fourth step of fatty acid breakdown? |
3-ketoacyl CoA is thiolysed by CoA to form Acyl CoA (which is shortened by 2 C) and Acetyl CoA |
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In each beta oxidation pathway reaction cycle, how many carbon atoms are released from the fatty acid? How many FADH2, NADH, and acetyl CoA molecules are formed? |
2 carbons are released
1 FADH2, NADH, and acetyl CoA are formed |
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Palmitate has 16 carbons. In the breakdown of this molecule, how many times is it cut and how many acetyl CoA molecules are formed? Why? |
It is cut 7 times forming 8 acetyl CoA molecules. This is because the last cut produces TWO acetyl CoA! |
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Palmitate has 16 carbons. In the breakdown of this molecule, how many times is it cut and what is the total energy yield? |
It is cut 7 times and produces 106 ATP molucules |
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How many ATPs per acetyl CoA in the citric acid cycle? |
10 |
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How are odd-numbered fatty acids broken down? |
An enzyme using vitamin B12 as a cofactor adds 1 carbon to the left over 3-carbon fragment |
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How are unsaturated fatty acids broken down? |
Enzymes move double bonds to be in register with the beta oxidation pathway |
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Excess acetyl CoA is converted into what 3 thing? What are these referred to as? |
1. Acetoacetate 2. 3-hydroxybutyrate 3. Acetone
They are referred to as KETONE BODIES |
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Ketone bodies are released into the blood for what purpose? |
To be used by some tissues as fuel instead of glucose |
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Ketone body synthesis takes place where? |
Mitochondria of the liver |
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In the formation of ketone bodies, what are the 5 steps? |
1. acetyl CoA to Acetoacetyl CoA 2. 3-hydroxy-3-methyl-glutaryl CoA 3. Acetoacetate 4. 3-hydroxybutyrate OR Acetone |
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Acetone, under starvation conditions, can be captured to do what? |
Synthesize glucose |
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3-Hydroxybutyrate can be oxidized to form what? |
TWO molecules of acetyl CoA (which goes on into the citric acid cycle) |
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Fatty acid synthesis occurs where and by how many carbon atoms at a time? |
In the cytoplasm by 2 carbon atoms at a time |
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What is the reducing agent of fatty acid synthesis? |
NADPH |
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What is the raw material of fatty acid synthesis? |
Acetyl CoA |
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How are acetyl CoA transported out of the mitochondria and into the cytoplasm so that fatty acids can be synthesized? |
Acetyl CoA is converted into citrate via condensation with oxaloacetate |
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What is the activated form of acetyl CoA? |
Malonyl CoA |
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Fatty acid synthesis starts with what? |
The carboxylation of acetyl CoA to malonyl CoA |
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What is the first committed step in fatty acid synthesis? |
Acetyl CoA with HCO3- to form Malonyl CoA |
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What are the 2 steps of malonyl CoA synthesis? |
1. Acetyl CoA carboxlylase containing BIOTIN hydrolyzes acetyl CoA 2. Activated CO2 group is then transferred to form malonyl CoA |
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Fatty acids are synthesized by the repetition of what 4 reactions? |
1. Condensation 2. Reduction 3. Dehydration 4. Reduction |
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The elongation phase of fatty acid synthesis in bacteria starts when acetyl CoA and malonyl CoA react with what? What is formed? |
A scaffold protein called acyl carrier protein (ACP) which forms acetyl ACP and malonyl ACP |
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In the 1st round of fatty acid synthesis, acetyl ACP and malonyl ACP form what by what process? |
Acetoacetyl ACP via CONDENSATION |
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Acetoacetyl ACP forms what by what process? What does it use? |
D-3-hydroxybutyrl ACP via REDUCTION using NADPH |
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D-3-hydroxybutyrl ACP forms what by what process? |
Crotonyl ACP via DEHYDRATION |
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Crotonyl ACP forms what by what process? What does it use? |
Butyryl ACP (4-carbons) via REDUCTION using NADPH |
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All the carbon atoms of fatty acids containing an EVEN number of carbon atoms are derived from what? |
Acetyl CoA |
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What two things start the 2nd round of fatty acid synthesis? |
Butyryl ACP (4 C) and Malonyl ACP |
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Half of the NADPH needed for fatty acid synthesis comes from where? Where does the other half come from? |
Half comes from the pentose phosphate pathway
Other half comes from mitochondrial export of acetyl CoA when Malate is converted into Pyruvate |
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After the formation of acetyl CoA, the resulting oxaloacetate is transported back into the mitochondria to form what? |
NADPH |
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Both triacylglycerol and phospholipid synthesis begin with what precursor? |
Phosphatidate |
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Phosphatidate is formed by the addition of what? |
2 fatty acids to glycerol 3-phosphate |
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Fats and phospholipids connect to glycolysis via what? |
Glycerol 3-phosphate |
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What are the 2 steps to make phosphatidate? Via what? |
1. Glycerol 3-phosphate to lysophosphatidate (via saturated acyl CoA) 2. Lysophosphatidate to phosphotidate (via unsaturated acyl CoA) |
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What are the 2 steps to make triacylglycerol? |
1. Phosphatidate to Diacylglycerol (DAG) (via hydrolysis) 2. DAG to triacylglycerol (via acyl CoA) |
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What are the 2 ways to make phospholipids? |
1. Biosynthesis from the activation of phosphatidase by CTP
2. Biosynthesis from the activation of the "head group" of DAG by CTP |
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Activation of phosphatidate to CDP-diacylglycerol is driven forward by what? |
The hydrolysis of pyrophosphate! |
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What are the 3 steps to form phosphatidylethanolamine? |
1. Ethanolamine to Phosphorylethanolamine 2. CDP-ethanolamine 3. Phosphatidylethanolamine |
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What is the role of CTP activation in the formation of phosphatidylethanolamine? |
CTP activates the alcohol phosphorylethanolamine to form CDP-ethanolamine |
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Phosphatidylcholine is synthesized from phosphatidylethanolamine how? Why? |
It is methylated with S-Adenosylme-thionine 3 times when dietary choline is insufficient. |
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Sphingolipids are synthesized from what? |
Ceramide |
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All 27 carbon atoms of cholesterol are derived from what? |
Acetyl CoA |
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What is the 3 stage synthetic process of cholesterol? |
1. Isopentenyl pyrophosphate synthesis 2. Condensation of 6 isopentenyl pyrophosyhtate molecules to form squalene 3. Squalene cyclizes into cholesterol |
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The formation of isopentenyl pyrophosphate from acetyl CoA starts with the formation of what? |
3-hydroxy-3-methylglutaryl CoA (HMG CoA) from acetyl CoA and acetoacetyl CoA |
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What is the role of HMG CoA Reductase? |
It catalyzes the formation of mevalonate from 3-hydroxy-3-methylglutaryl CoA in Cholesterol formation. |
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What is mevalonate? |
An important intermediate formed from HMG CoA in the formation of cholesterol |
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What is the committed step of cholesterol biosynthesis? |
Formation of mevolonate |
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In the Cytosol, HMG-CoA forms what? |
Cholesterol |
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In the mitochondria, HMG-CoA forms what? |
Ketone bodies |
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What is the building block of cholesterol biosynthesis? |
Isoprene |
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Mevalonate is converted into what in 3 consecutive ATP reactions? |
3-isopentenyl pyrophosphate |
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Squalene (30 C) is synthesized from 6 molecules of what? |
Isopentenyl pyrophosphate (5 C) |
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What must happen to isopentenyl pyrophosphate before condensation can take place? |
It must isomerize into dimethylallyl pyrophosphate |
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Farnesyl pyrophosphate is converted into what using what? |
Squalene using farnesyl pyrophosphate and NADPH |
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Cholesterol and other lipids in excess of those needed by the liver are exported in what form? |
Low density lipoproteins (LDLs) |
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Liver and peripheral-tissue cells take up LDL by what? |
Receptor-mediated endocytosis |
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What does the LDL receptor do? |
Binds LDL and mediates its entry into the cell |
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The absence of LDL receptors in the homozygous form of familial hypercholesterolemia leads to what? |
Elevated plasma levels of LDL cholesterol and the deposition of cholesterol in blood vessels leading to heart attacks. |
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Homozygous familial hypercholesterolemia can only be treated by what? |
A liver transplant |
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What is the major carrier of cholesterol in the blood? |
Low-density lipoprotein |
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What 4 things surround the LDL? |
1. Unesterified cholesterol 2. Phospholipid 3. Cholesteryl ester 4. Apoprotein B-100 |
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What is the purpose of Apoprotein B-100? |
Directs LDL to the proper cells |
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What is the role of LDL? |
Transport cholesterol to peripheral tissues and regulate de novo cholesterol synthesis at these sites |
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What are the 3 steps to receptor-mediated endocytosis of LDL? |
1. LDL binds to receptor protein on cell surface 2. Cell internalizes the receptor-LDL complex 3. LDL is hydrolyzed in lysosomes |
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What are the 2 steps to amino acid catabolism? |
1. Remove alpha-amino group 2. Deliver carbon "skeleton" to glycolysis and/or citric acid cycle for oxidation |
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The carbon skeleton of alanine (once the NH4 is removed) is what? |
Pyruvate! |
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The alpha amino group of many amino acids is transferred to what to form what? What happens next? |
To alpha-ketoglutarate to form glutamate which is then oxidatively deaminated to yield ammonium (NH4+) |
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What catalyzes the transfer of an alpha-amino group from an alpha amino acid to an alpha-ketoacid? |
Aminotransferases! |
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What does aspartate aminotransferase do? |
Transfers the alpha-amino group of aspartate to alpha-ketoglutarate to form oxaloacetate and glutamate AND VICE VERSA |
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What happens to the nitrogen atom that is transferred to alpha-ketoglutarate in the transamination reaction? |
It is converted into free ammonium ion by the oxidative deamination of glutamate, regenerating alpha-ketoglutarate |
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How is nitrogen removed from glutamate? |
Glutamate dehydrogenase |
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Which amino acids can be directly converted into ammonium ion? By what? |
Serine and threonine by serine and threonine dehydratase, respectively. |
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During fasting or exercise, muscle can use amino acids as fuel, exporting nitrogen as what? |
Alanine |
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If pyruvate is exported as alanine without reoxidizing NADH by forming lactate, how can glycolysis continue? |
NADH is oxidized by the citric acid cycle |
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What 6 amino acid carbon skeletons can feed into pyruvate of the citric acid cycle? |
1. Alanine 2. Cysteine 3. Glycine 4. Serine 5. Threonine 6. Tryptophan |
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What 2 amino acid carbon skeletons can feed into oxaloacetate of the citric acid cycle? |
1. Asparagine 2. Aspartate |
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What 3 amino acid carbon skeletons can feed into fumarate of the citric acid cycle? |
1. Aspartate 2. Phenylalanine 3. Tyrosine |
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What 4 amino acid carbon skeletons can feed into Succinyl CoA of the citric acid cycle? |
1. Isoleucine 2. Methionine 3. Threonine 4. Valine |
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What 5 amino acid carbon skeletons can feed into alpha-ketoglutarate of the citric acid cycle? |
1. Arginine 2. Glutamate 3. Glutamine 4. Histidine 5. Proline |
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Where do the nitrogen atoms of urea come from? Where does the carbon atom come from? |
One N comes from aspartate, the other comes from free ammonium ions
The C comes from HCO3- (derived from the hydration of CO2) |
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The urea cycle begins in the mitochondria with the coupling of what 2 things to form what? |
Free NH4+ and HCO3- to form carbamoyl phosphate |
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What are the 3 steps to form carbamoyl phosphate? What catalyzes all of these reactions? |
1. Bicarbonate to carboxyphosphate 2. Carboxyphosphahte to Carbamic acid 3. Carbamic acid to carbamoyl phosphate
Catalyzed by carbamoyl phosphate synthetase |
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The carbamoyl group of carbamoyl phosphate is transferred to what to form what? What catalyzes it? |
Transferred to ornithine to form citrulline via ornithine transcarbamoylase |
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What are the 4 steps to the Urea Cycle starting with Citrulline? |
1. Citrulline (w/ Aspartate) forms Argininosuccinate 2. Argininosuccinate to Arginine (releasing Fumarate) 3. Arginine to Ornithine (releasing UREA) 4. Ornithine (w/ Carbamoyl Phosphate) to Citrulline |
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Ammonia is carried on what activated molecule that then goes on to form carbamoyl phosphate? |
Bicarbonate! |
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Urea is produced by removing Nitrogen from what? |
Arginine |
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TRUE OR FALSE: The urea cycle only spans one compartment, the mitochondrial matrix. |
FALSE. It spans both the cytoplasm and the mitochondrial matrix |
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Fumarate from the urea cycle can go on to make what? What can it also do? |
Glucose via gluconeogenesis
OR
it can enter the citric acid cycle |
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Cytoplasmic malate enters the matrix and exits as what? |
Aspartate |
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What 2 things can happen to oxaloacetate? |
1. Converted into glucose via gluconeogenesis 2. Transaminated to aspartate |
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The carbon skeletons of amino acids are funneled into what 7 molecules? |
1. Pyruvate 2. Acetyl CoA 3. Acetoacetyl CoA 4. Alpha-ketoglutarate 5. Succinyl CoA 6. Fumarate 7. Oxaloacetate |
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Amino acids that are degraded into acetyl CoA or acetoacetyl CoA are called what? Why? |
Ketogenic amino acids because they can give rise to ketone bodies or fatty acids and CANNOT be used to synthesize glucose |
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Which 2 amino acids are SOLELY ketogenic? |
Leucine and lysine |
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Which 5 amino acids are both ketogenic and glucogenic? |
1. Threonine 2. Isoleucine 3. Phenylalanine 4. Tryptophan 5. Tyrosine |
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Atmospheric nitrogen is converted into what? |
Ammonia |
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Ammonium + alpha-ketoglutarate --> ??? |
Glutamate |
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Glutamate + alpha-keto acid --> ??? |
Alpha-ketoglutarate + amino acid |
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What is biological nitrogen fixation? |
Conversion of atmospheric nitrogen into |
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What is industrial nitrogen fixation? |
Conversion of atmospheric nitrogen into ammonia carried out at 500 C and 300 atm pressure using Fe catalyst (Haber process) |
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Biological nitrogen fixation is catalyzed by what? |
Nitrogenase |
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Glutamate synthesis is catalyzed by what? |
Glutamate dehydrogenase (which is the same enzyme used in both degadation and biosynthesis of amino acids) |
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How is glutamate synthesized? |
Alpha-ketoglutarate and ammonium is dehydrolyzed then protonated to form glutamate. |
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What are the 2 basic steps to form glutamine? |
1. Glutamate to acyl-phosphate intermediate 2. acyl-phosphate intermediate to glutamine |
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Glutamate is converted into acyl-phosphate intermediate using what? |
ATP |
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Acyl-phosphate intermediate is converted into glutamine using what? What is released? |
NH3 which releases an inorganic phosphate |
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What 3 things are formed from 3-phosphoglycerate? |
1. Serine 2. Cysteine 3. Glycine |
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What is tetrahydrofolate? |
Coenzyme essensial for the synthesis of amino acids that is a carrier of activated 1-carbon units. |
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What are the 3 groups of tetrahydrofolate? |
1. a substituted pteridine 2. p-aminobenzoate 3. 1+ glutamate residue chain |
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In the formation of glycine, the side chain methylene group of serine is transferred to what? |
Tetrahydrofolate, a carrier of one-carbon units |
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What are the 9 essential (CANNOT be synthesized) amino acids? |
1. Histidine 2. Isoleucine 3. Leucine 4. Lysine 5. Methionine 6. Phenylalanine 7. Threonine 8. Tryptophan 9. Valine |
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TRUE OR FALSE: Non-essential amino acids have complex pathways. |
FALSE. They have simple pathways. |
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Alpha-ketoglutarate goes to glutamate which goes to what 3 amino acids? |
1. Glutamine 2. Proline 3. Arginine |
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Pyruvate makes what 3 amino acids? |
1. Alanine 2. Valine 3. Leucine |
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Oxaloacetate goes to aspartate which can make what 5 amino acids? |
1. Asparagine 2. Methionine 3. Threonine 4. Isoleucine (From threonine) 5. Lysine |
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What are the 3 steps to make serine? |
1. 3-phosphoglycerate to 3-phosphohydroxypyruvate 2. 3-phosphoserine 3. Serine |
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How is 3-phosphoglycerate converted into 3-phosphohydroxypyruvate? |
It's oxidized via NAD+ (forming NADH) |
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How is 3-phosphohydroxypyruvate converted into 3-phosphoserine? |
Transamination via glutamate (forming alpha-ketoglutarate) |
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How is 3-phosphoserine converted into serine? |
Hydrolysis! |
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The one-carbon group carried by tetrahydrofolate is bonded to what? |
Tetrahydrofolate's N-5 or N-10 or to both! |
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The most reduced form of tetrahydrofolate carries what? What does the intermediate carry? |
A methyl group
The intermediate carries a methylene group |
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The most oxidized form of tetrahydrofolate can carry what 3 things? |
1. Formyl 2. Formimino 3. Methenyl groups |
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The fully oxidized one carbon unit, CO2, is carried by what rather than by tetrahydrofolate? |
Biotin! |
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What is the major donor of methyl groups? |
S-adenosylmethionine (SAM) |
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SAM is synthesized by what? |
The transfer of an adenosyl group from ATP to the sulfur atom of methionine |
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How is serine converted into glycine? |
Tetrahydrofolate removes the side chain of serine to produce glycine and N5,N10-methylenetetrahydrofolate. |
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Along with NADPH, N5,N10-methylenetetrahydrofolate is converted into what for further methylation reactions? |
N5-methyltetrahydrofolate |
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Methionine + ATP ---> ??? |
S-Adenosylmethionine + Pi + PPi |
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Methionine is regenerated from what? |
N5-Me-THF and Homocysteine |
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Serine inhibits its own synthesis at what point? |
The oxidation reaction between 3-phosphoglycerate and NAD+ |
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What are the 2 pathways of nucleotide biosynthesis? |
1. De novo 2. Salvage |
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What is De novo? |
Nucleotide synthesis built up from small precursors |
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What is Salvage? |
Nucleotide recovery from other reactions and added to ribose |
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In pyrimidine synthesis, N1 and C4,5,6 come from where? |
Aspartate |
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In pyrimidine synthesis, C2 and N3 come from where? |
Carbamoyl phosphate |
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What are the 3 precursors of pyrimidine rings? |
1. Bicarbonate 2. Aspartic acid 3. Ammonia |
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What are the 3 steps of carbamoyl phosphate synthesis? Where do all three steps occur? |
1. Bicarbonate to Carboxyphosphate (ATP) 2. Carboxyphosphate to Carbamic Acid (NH3 from Gln) 3. Carbamic Acid to Carbamoyl Phosphate (ATP)
All three steps occur on carbamoyl phosphate synthase! |
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In pyrimidine nucleotide synthesis, what are the 3 acts? |
1. Pyrimidine ring formation 2. Attachment to ribose phosphate 3. 2' deoxy |
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The Urea cycle uses which carbamoyl phosphate synthase? |
CPS-1: NH3 from ammonium |
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Pyrimidine synthesis uses which carbamoyl phosphate synthase? |
CPS-2: NH3 from glutamine |
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What is 5-phosphoribosyl-1-pyrophosphate (PRPP)? How is it synthesized? |
Activated ribose for base attachment that is synthesized from ribose 5-phosphate (from the pentose phosphate pathway). |
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Deoxyribonucleotide synthesis is catalyzed by what? |
Ribonucleotide reductase |
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In deoxyribonucleotide synthesis, deoxyribonucleotides are made via what mechanism? |
Cysteine free radical mechanism |
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Oxidized cysteines are reduced by what in Deoxyribonucleotide synthesis? |
NADPH |
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What is the role of tetrahydrofolate in the formation of thymidylate? |
N5,N10-methylenetetrahydrofolate is the methyl donor for deoxyuridine monophonphate (dUMP) that creates thymidylate (TMP) and dihydrofolate. |
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Dihydrofolate, which is made during thymidylate synthesis, is reduced to form what? |
Tetrahydrofolate via dihydrofolate reductase and NADPH |
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Carbamoyl phosphate is converted into what in nucleotide synthesis? |
Carbamoylaspartate via aspartate and aspartate transcarbamoylase |
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Carbamoylaspartate is converted into what? |
Orotate (NADH is formed as well) |
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Orotate reacts with what to form what? |
Reacts with PRPP to form orotidylate, a pyrimidine nucleotide |
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Orotidylate is then decarboxylated via what to form what? |
Uridylate (Uridine Monophosphate, UMP) via orotidylate decarboxylase |
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How is cytidine monophosphate (CMP) formed? |
UMP is converted to UTP via ATP, then UTP's carbonyl group is replaced by an amino group donated by glutamine to form CTP. |