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56 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Galactose → Galactose-1-phosphate
What enzyme? |
Galactokinase
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Deficiency of Galactokinase causes what disorder?
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(mild galactosemia)
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Galactose-1-phosphate → Glucose-1-phosphate
What enzyme? |
Galactose-1-phosphate uridyltransferase
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Deficiency of Galactose-1-phosphate uridyltransferase causes what disorder?
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(severe galactosemia)
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Glucose → Glucose-6-phosphate
What enzyme? |
Hexokinase / Glucokinase
(rate limiting enzyme in Glycolysis) |
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Glucose-6-phosphate → Glycose
What enzyme? |
Glucose-6-phosphatase
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Deficiency of Glucose-6-phosphatase causes what disease?
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(von Gierke's)
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Glucose-6-phosphate → 6-phosphogluconolactone
What enzyme? |
Glucose-6-phosphate dehydrogenase
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Deficiency of Glucose-6-phosphate dehydrogenase causes what disorder?
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(G6PD)
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Ribulose-5-phosphate ↠ Fructose-6-phosphate
What enzyme? |
Transketolase
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Transketolase requires what cofactor?
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Thiamine
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Fructose-6-phosphate → Fructose-1,6-bisphosphate
What enzyme? |
Phosphofructokinase
(rate limiting enzyme) |
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Fructose-1,6-bisphosphate → Fructose-6-phosphate
What enzyme? |
Fructose-1,6-bisphosphatase
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Fructose → Fructose-1-phosphate
What enzyme? |
Fructokinase
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Deficiency of Fructokinase causes what disorder?
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(essential fructosuria)
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Fructose-1-phosphate → DHAP
Fructose-1-phosphate → Glyceraldehyde What enzyme? |
Aldolase B
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Deficiency of Aldolase B causes what disorder?
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(fructose intolerance)
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Phosphoenolpyruvate (PEP) → Pyruvate
What enzyme? |
Pyruvate kinase
(rate limiting enzyme) |
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Pyruvate → Acetyl CoA
What enzyme? |
Pyruvate dehydrogenase
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Pyruvate dehydrogenase requires what cofactor?
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Thiamine
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HMG-CoA → Mevalonate
What enzyme? |
HMG-CoA reductase
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Pyruvate → Oxaloacetate
What enzyme? |
Pyruvate carboxylase
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Pyruvate carboxylase requires what cofactor?
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Biotin
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Oxaloacetate → Phosphoenolpyruvate (PEP)
What enzyme? |
Phosphoenolpyruvate carboxykinase
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Acetyl CoA + Oxaloacetate → Citrate
What enzyme? |
Citrate synthase
(rate limiting enzyme) |
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α-ketoglutarate → Succinyl-CoA
What enzyme? |
α-ketoglutarate dehydrogenase
(rate limiting enzyme) |
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α-ketoglutarate dehydrogenase requires what cofactor?
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Thiamine
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Ornithine + Carbamoyl phosphate → Citruline
What enzyme? |
Ornithine transcarbamylase
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Name the irreversible enzymes of Gluconeogenesis.
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1. Pyruvate carboxylase (mitochondria)
2. PEP carboxykinase 3. Fructose-1,6-bisphosphatase 4. Glucose-6-phosphatase (2., 3. & 4. in the cytosol) |
These enzymes are found only in liver, kidney, intestinal epithelium. (NOT found in the muscle)
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What metabolic processes occur in the mitochondria?
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1. Fatty acid oxidation (β-oxidation)
2. Acetyl-CoA production 3. Krebs cycle |
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What metabolic processes occur in the cytoplasm?
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1. Glycolysis
2. Fatty acid synthesis 3. HMP shunt 4. Protein systhesis (RER) 5. Steroid systhesis (SER) |
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What metabolic processes occur in both mitochondria and cytoplasm?
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1. Gluconeogenesis
2. Urea cycle 3. Heme synthesis |
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Name the irreversible enzymes of Glycolysis.
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1. Hexokinase / Glucokinase
2. Phosphofructokinase-1 3. Pyruvate kinase 4. Pyruvate dehydrogenase |
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Rate-determining enzyme of De novo pyrimidine synthesis.
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Aspartate transcarbamylase (ATCase)
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Rate-determining enzyme of De novo purine synthesis.
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Glutamine-PRPP amidotransferase
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Rate-determining enzyme of Glycolysis.
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Phosphofruktokinase-1 (PFK-1)
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Rate-determining enzyme of Gluconeogenesis.
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Pyruvate carboxylase
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Rate-determining enzyme of TCA cycle.
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Isocitrate dehydrogenase
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Rate-determining enzyme of Glycogen synthesis.
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Glycogen synthase
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Rate-determining enzyme of Glycogenolysis.
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Glycogen phosphorylase
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Rate-determining enzyme of HMP shunt.
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Glucose-6-phosphate dehydrogenase (G6PD)
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Rate-determining enzyme of Fatty acid synthesis.
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Acetyl-CoA carboxylase (ACC)
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Rate-determining enzyme of Fatty acid oxidation.
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Carnitine acyltransferase I
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Rate-determining enzyme of Ketogenesis.
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HMG-CoA synthase
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Rate-determining enzyme of Cholesterol synthesis.
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HMG-CoA reductase
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Rate-determining enzyme of Heme synthesis.
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ALA synthase
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Rate-determining enzyme of Urea cycle.
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Carbamoyl phosphate synthase I
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Universal electron acceptors:
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Nicotinamides (NAD⁺, NADP⁺)
Flavin (FAD⁺) NAD⁺ is generally used in catabolic processes (to carry reducing equivalents away as NADH). NADPH is used in anabolic processes (as a supply of reducing equivalents). |
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NADPH is used in?
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1. Anabolic processes
2. Respiratory burst 3. P-450 |
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NADPH ⇌ NADP⁺
What enzyme? |
NADPH oxidase
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NADPH oxidase deficiency causes causes what disorder?
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Chronic Granulomatous Disease (CGD)
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Patients with NADPH oxidase deficiency are more susceptible to infections with what organisms?
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Catalase positive organisms
(S. aureus, E. coli, Aspergillus and Klebsiella). |
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Patients with myeloperoxidase deficiency are more susceptible to infections with what organisms?
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Catalase negative organisms
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Deficiency of Galactose-1-phosphate uridyltransferase (GALT) causes what disorder?
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Galastosemia
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Symptoms: cataracts, hepatosplenomegaly, mental retardation.
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Deficiency of Pyruvate dehydrogenase causes what problem?
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Lactic acidosis
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Backup of substrate.
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G6PD deficiency
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Hemolytic amenia
Heinz bodies Bite cells X-linked recessive more prevalent among African Americans ↑ malarial resistance |
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