Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
Fatty acids added to ___ to make a TAG |
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Definition
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Term
|
Definition
1) Pancreatic, 2) lipoprotein, 3) hormone-sensitive |
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Term
Pancreatic lipase: secreted into what |
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Definition
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|
Term
Pancreatic lipase: products |
|
Definition
2-monoglyceride & 2 free FA's |
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Term
Intestinal TG's destined for being in what |
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Definition
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Term
|
Definition
TG core with protein+phospholipid shell |
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Term
Lipoprotein lipase: location |
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Definition
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|
Term
Hormone-sensitive lipase: location |
|
Definition
Tissues (e.g., testes & adipose) |
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Term
Hormone-sensitive lipase: products |
|
Definition
TAG → DAG → MAG → glycerol |
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|
Term
Hormone-sensitive lipase: activation through what process |
|
Definition
Glucagon or EPI through PKA |
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|
Term
Hormone-sensitive lipase: where does glycerol go |
|
Definition
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|
Term
Hormone-sensitive lipase: routes of free FA's |
|
Definition
1) Mitochondria, 2) hitchin' a ride on albumin in the blood |
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Term
Hormone-sensitive lipase: destinations of free FA's (2) |
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Definition
1) Hepatic, 2) extrahepatic |
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Term
Hormone-sensitive lipase: hepatic destination produces what |
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Definition
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|
Term
Hormone-sensitive lipase: extrahepatic destination produces what |
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Definition
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|
Term
How does a FA on albumin cross the plasma membrane |
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Definition
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|
Term
Free FA's bind to what after crossing plasma membrane |
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Definition
Fatty acyl binding protein |
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Term
Purpose of fatty acyl binding protein (FABP) |
|
Definition
Keeps FA's from aggregating in the cell |
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|
Term
Activation of FA's: reaction |
|
Definition
FA + CoA + ATP → FA-CoA + AMP + PPi |
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|
Term
Activation of FA's: enzyme |
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Definition
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|
Term
Activation of FA's: routes for activated FA's (4) |
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Definition
1) β-oxidation, 2) phospholipids, 3) sphingolipids, 4) triglycerides |
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Term
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Definition
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|
Term
CoA active site is what amino acid |
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Definition
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|
Term
Acyl carnitine transport: how many pools of CoA |
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Definition
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|
Term
Acyl carnitine transport: what swaps CoA for carnitine |
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Definition
Carnitine palmitoyl transferase I |
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|
Term
Acyl carnitine transport: what swaps carnitine for CoA |
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Definition
Carnitine palmitoyl transferase II |
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Term
Acyl carnitine transport: carnitine palmitoyl transferase I in which membrane |
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Definition
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|
Term
Acyl carnitine transport: carnitine palmitoyl transferase II in which membrane |
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Definition
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|
Term
Acyl carnitine transport: what moves acyl-carnitine across inner membrane |
|
Definition
Acyl carnitine transporter (translocase) |
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|
Term
Acyl carnitine transport: how long of an FA |
|
Definition
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|
Term
Long-chain fatty acids into mitochondria how |
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Definition
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|
Term
Short- & medium-chain fatty acids into mitochondria how |
|
Definition
Dunno, but not acyl carnitine transport |
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|
Term
Very-long-chain fatty acids go where |
|
Definition
Peroxisomes first, then mitochondria |
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|
Term
β-oxidation: ACoA DH equivalent in TCAC |
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Definition
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|
Term
β-oxidation: succininc DH in TCAC equivalent in β-ox |
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Definition
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|
Term
β-oxidation: enoyl-CoA hydratase equivalent in TCAC |
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Definition
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|
Term
β-oxidation: fumarase in TCAC equivalent in β-ox |
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Definition
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|
Term
β-oxidation: 3-hydroxyacyl-CoA DH equivalent in TCAC |
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Definition
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|
Term
β-oxidation: malic DH in TCAC equivalent in β-ox |
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Definition
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|
Term
β-oxidation: which uses FAD |
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Definition
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|
Term
β-oxidation: which uses FADH2 |
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Definition
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|
Term
β-oxidation: which uses NAD |
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Definition
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|
Term
β-oxidation: which uses NADH |
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Definition
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|
Term
β-oxidation: which uses H2O |
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Definition
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|
Term
β-oxidation: palmitic acid into what products |
|
Definition
8 ACoA + 7 NADH + 7 FADH2 |
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|
Term
β-oxidation: palmitic acid into ATP |
|
Definition
8 ACoA=96 ATP + 7 NADH=21 ATP + 7 FADH2=14 ATP - 2 ATP for activation = 129 ATP |
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|
Term
ATP shortage in β-oxidized cells leads to (4) |
|
Definition
1) Hypoglycemia, 2) hypoketonemia, 3) hyperuricemia, 4) carnitine deficiency |
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|
Term
Which is more fatal: CPT I or CPT II |
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Definition
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|
Term
Which affects the liver: CPT I or CPT II |
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Definition
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Term
Which affects the muscle: CPT I or CPT II |
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Definition
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Term
|
Definition
Carbohydrate diet low in fat |
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|
Term
MCAD deficiency treatment |
|
Definition
Carbohydrate diet with short-chain FA's |
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Term
|
Definition
Fatal defect in peroxisome biogenesis |
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Term
X-linked adrenoleukodystrophy |
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Definition
Defect in very-long FA activation |
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|
Term
Methods to measure ketones (2) |
|
Definition
1) Dipstick, 2) direct for β-hydroxybutyrate |
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|
Term
Advantages of ketones (3) |
|
Definition
Shunts away 1) ACoA, 2) H+, 3) electrons |
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|
Term
Conditions when ketones are more heavily produced (3) |
|
Definition
1) Excess ACoA (need CoA), 2) NAD insufficiency, 3) OAA insufficiency |
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|
Term
|
Definition
1) Acetoacetate, 2) β-hydroxybutyrate, 3) acetone |
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|
Term
NAD:NADH ratio matches which ratio |
|
Definition
Acetoacetate to β-hydroxybutyrate |
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|
Term
Synthesis of acetoacetate |
|
Definition
2 ACoA → AACoA; AACoA + ACoA → HMG-CoA + CoA; HMG-CoA → acetoacetate + ACoA |
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|
Term
Synthesis of β-hydroxybutyrate |
|
Definition
Acetoacetate + NADH → β-hydroxybutyrate + NAD |
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|
Term
Typical ratio of β-hydroxybutyrate to acetoacetate |
|
Definition
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|
Term
|
Definition
|
|
Term
|
Definition
Decarboxylation of acetoacetate: acetoacetate → acetone + CO2 |
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|
Term
Enzyme in non-heptic for ketone utilization and what reaction |
|
Definition
Aceto-acetate-succinyl CoA transferase: β-hydroxybutyrate + SCoA → AACoA + succinate [finally: AACoA → 2 ACoA] |
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|
Term
Enzyme that makes bicarbonate |
|
Definition
|
|
Term
Cofactor with carbonic anhydrase |
|
Definition
|
|
Term
HH equation for bicarbonate buffer |
|
Definition
pH=6.1 + log(HCO3/(0.03 * PCO2)) |
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|
Term
|
Definition
Organic acid into blood → CO2 blown off → ↑anion gap |
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|
Term
Kassmaul respiration associated with |
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Definition
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|
Term
ACoA leaves mitochondria in what way |
|
Definition
Exported as citrate through tricarboxylic transporter |
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|
Term
Cytosolic citrate total reaction |
|
Definition
Citrate + ATP + NADP → MalCoA + Pyr + ADP + Pi + CO2 + NADPH |
|
|
Term
Vitamin required for cytosolic citrate handling |
|
Definition
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|
Term
|
Definition
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|
Term
ACoA carboxylase: active or inactive when polymerized |
|
Definition
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|
Term
ACoA carboxylase: active or inactive when phosphorylated |
|
Definition
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|
Term
ACoA carboxylase: polymerized by |
|
Definition
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|
Term
ACoA carboxylase: depolymerized by (2) |
|
Definition
1) FA's, 2) phosphorylation |
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|
Term
ACoA carboxylase: phosphorylated by |
|
Definition
|
|
Term
ACoA carboxylase: dephosphorylated by |
|
Definition
|
|
Term
ACoA carboxylase: ultimately, glucagon or EPI: activates or inactivates |
|
Definition
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|
Term
|
Definition
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|
Term
Overall reaction for creating palmitate |
|
Definition
1 ACoA + 7 MalCoA + 14 NADPH → palmitate + 14 NADP + 8 CoA + 7 CO2 |
|
|
Term
Net NADPH required for palmitate production from citrate |
|
Definition
14 for synthesis less 8 from citrate = 6 total NADPH required per palmitate |
|
|
Term
Enzymatic sites of FA synthesis (2) |
|
Definition
1) Condensing enzyme, 2) acyl carrier protein (ACP) |
|
|
Term
Which site first binds ACoA and MalCoA |
|
Definition
|
|
Term
Which site holds elongating FA |
|
Definition
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|
Term
Desaturation by what enzymes (3) |
|
Definition
1) Stearoyl CoA desaturase, 2) FADS1, 3) FADS2 |
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|
Term
Stearoyl CoA desaturase desaturates where |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
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|
Term
Which is a Δ9 desaturase: stearoyl CoA desaturase, FADS1, FADS2 |
|
Definition
|
|
Term
Which is a Δ5 desaturase: stearoyl CoA desaturase, FADS1, FADS2 |
|
Definition
|
|
Term
Which is a Δ6 desaturase: stearoyl CoA desaturase, FADS1, FADS2 |
|
Definition
|
|
Term
Essential fatty acids (2; classes not specific molecules) |
|
Definition
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|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Root cause of ketoacidosis |
|
Definition
Increased glucagon = hypergluconemia |
|
|
Term
Glucagon inhibits what [as it relates to ketoacidosis] |
|
Definition
|
|
Term
Inhibition of MalCoA does what (2) |
|
Definition
1) Shuts down FA synthesis, 2) activates carnitine acyl transferase |
|
|
Term
ACoA, NAD, FAD conditions leading to ketone production |
|
Definition
Excess ACoA, low NAD, low FAD |
|
|
Term
Excess ketone production causes ketoacidosis how |
|
Definition
Overloads bicarbonate buffer system |
|
|
Term
Chronic hyperglycemia hypothesized to cause what |
|
Definition
|
|
Term
Glycation of what structures (2) |
|
Definition
1) α-Hb (HBA) to HbA1c, 2) membrane proteins |
|
|
Term
HbA1c: 1% = ___ mg/dL glucose |
|
Definition
1% = 35 mg/dL [so normal is about 6%] |
|
|
Term
Consequences of advanced glycation endproducts (6) |
|
Definition
1) Neuropathy, 2) ulcers, 3) amputation, 4) retinopathy, 5) heart disease, 6) infections |
|
|
Term
|
Definition
Inhibits gluconeogensis [for type 2 DM] |
|
|
Term
|
Definition
Increase insulin secretion [for type 2 DM] |
|
|
Term
|
Definition
Increase insulin sensitivity [for type 2 DM] |
|
|
Term
|
Definition
Lower carbohydrate intake by intestines [for type 2 DM] |
|
|
Term
|
Definition
Reverse advanced glycation endproducts [for type 1 & 2 DM] |
|
|
Term
Which deficiency leads to acidosis: γ-glutamylcysteine synthetase or glutathione synthetase |
|
Definition
|
|
Term
Which deficiency does not lead to acidosis: γ-glutamylcysteine synthetase or glutathione synthetase |
|
Definition
γ-Glutamylcysteine synthetase |
|
|
Term
Which amino acids are only ketogenic (2) |
|
Definition
|
|
Term
How many ATP per amino acid from intestine lumen into blood |
|
Definition
|
|
Term
|
Definition
|
|
Term
Glutamyl cyle: ATP-requiring enzymes (3) |
|
Definition
1) 5-Ketoprolinase, 2) γ-glutamylcysteine synthetase, 3) glutathione synthetase |
|
|
Term
|
Definition
|
|
Term
More nitrogen out than in: positive or negative nitrogen balance |
|
Definition
Negative nitrogen balance |
|
|
Term
More nitrogen in than out: positive or negative nitrogen balance |
|
Definition
Positive nitrogen balance |
|
|
Term
Starvation: positive or negative nitrogen balance |
|
Definition
Negative nitrogen balance |
|
|
Term
Main AA's for transporting ammonia (2) |
|
Definition
|
|
Term
Transamination works on all AA's but (2) |
|
Definition
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|
Term
|
Definition
|
|
Term
|
Definition
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|
Term
|
Definition
|
|
Term
Transamination requires what vitamin |
|
Definition
|
|
Term
Glutamate DH inhibited by |
|
Definition
|
|
Term
Amino acid oxidase reaction |
|
Definition
AA + FAD → αKA + NH4 + FADH2 |
|
|
Term
What re-oxidizes FADH2 for amino acid oxidases |
|
Definition
Molecular oxygen [to H2O2] |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Enzyme used in kidneys to regulate body pH |
|
Definition
|
|
Term
Urea: first NH3 from ___, second NH3 from ___ |
|
Definition
|
|
Term
Energetic cost of urea cycle |
|
Definition
1 ATP: 4 ATP in but get out 1 NADH (=3 ATP) |
|
|
Term
Mitochondrial enzymes of urea cycle (2) |
|
Definition
|
|
Term
Cytosolic enzymes of urea cycle (3) |
|
Definition
1) Argininosuccinate synthetase, 2) argininosuccinase, 3) arginase |
|
|
Term
|
Definition
Activated by N-acetylglutamate |
|
|
Term
Which urea cycle enzyme is X-linked |
|
Definition
|
|
Term
Hyperammonemia type 1 from what enzyme deficiency |
|
Definition
|
|
Term
Congenital hyperammonemia type 2 from what enzyme deficiency |
|
Definition
|
|
Term
Citrullinemia from what enzyme deficiency |
|
Definition
Arginonsuccinate synthetase |
|
|
Term
Arginonsuccinacidemia fom what enzyme deficiency |
|
Definition
|
|
Term
Treat congenital hyperammonemia type 2 with what (2) |
|
Definition
1) Sodium benzoate & sodium phenylbutyrate, or 2) citrulline |
|
|
Term
Treat hyperammonemia type 1 with what (2) |
|
Definition
1) Sodium benzoate & sodium phenylbutyrate, or 2) citrulline |
|
|
Term
Sodium benzoate & sodium phenylbutyrate treats what enzyme deficiencies (2) |
|
Definition
1) CPS1, 2) OTC [those that get N into urea cycle] |
|
|
Term
for hyperammonemias, feeding citrulline works how |
|
Definition
Shunts nitrogen from Asp into urine |
|
|
Term
In general, what to feed for urea cycle defects (3) |
|
Definition
1) Na benzoae/Na phenylbutyrate, 2) Arg, 3) citrulline [substrates that feed past enzyme block] |
|
|
Term
|
Definition
|
|
Term
Nitric oxide stimulates what |
|
Definition
|
|
Term
What's special about guanylate cyclase from NO stimulation |
|
Definition
|
|
Term
Cofactors for NO synthase |
|
Definition
|
|
Term
NO synthase: needs NADPH or NADP or NADH or NAD |
|
Definition
|
|
Term
Viagra works by requiring what NO reaction to take place first |
|
Definition
NOS to make NO to activate heme-dependent guanylate cyclase to form cGMP |
|
|
Term
|
Definition
|
|
Term
AA direct transamination to TCAC/glycolysis intermediates (5) |
|
Definition
|
|
Term
AA 3-carbon to pyruvate (3) |
|
Definition
|
|
Term
AA 5-carbon in a row to αKG (6) |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Branched AA catabolic reactions (3) |
|
Definition
1) Transamination, 2) oxidative decarboxylation, 3) partial/complete β-oxidation |
|
|
Term
PCoA → SCoA reactions (2) |
|
Definition
1) PCoA carboxylase, 2) methylmalonyl CoA mutase |
|
|
Term
Branched AA catabolism: transamination cofactor |
|
Definition
|
|
Term
Branched AA catabolism: oxidative decarboxylation cofactors |
|
Definition
1) NAD, 2) FAD, 3) lipoic acid, 4) TPP, 5) CoA |
|
|
Term
PCoA carboxylase cofactor |
|
Definition
|
|
Term
Methylmalonyl CoA mutase cofactor |
|
Definition
|
|
Term
Active form of cobalamine |
|
Definition
|
|
Term
|
Definition
|
|
Term
Required protein for uptake of B12 |
|
Definition
|
|
Term
Intrinsic factor used for |
|
Definition
|
|
Term
Reasons to love cysteine (4) |
|
Definition
1) Disulfide bonds, 2) glutathione, 3) cysteine proteases, 4) reduction reactions |
|
|
Term
Reasons to love methionine (3) |
|
Definition
1) Essential AA, 2) START codon, 3) donor of methyl group |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Reaction classes folic acid is involved with (3) |
|
Definition
1) Methylation, 2) hydroxymethylation, 3) formylation |
|
|
Term
AA contained within folic acid |
|
Definition
|
|
Term
# of "copies" of a particular AA in folic acid |
|
Definition
|
|
Term
Reduction reactions of folate |
|
Definition
|
|
Term
|
Definition
1) Serine, 2) glycine, 3) histidine, 4) formate |
|
|
Term
THF irreversible reaction |
|
Definition
Irreversible when forming methyl-THF |
|
|
Term
Nonketotic hyperglycinemia |
|
Definition
Deficiency in glycine cleavage enzyme |
|
|
Term
Acute cyanide poisoning treated with |
|
Definition
|
|
Term
Megaloblastic/macrocytic anemia |
|
Definition
Decreased #, ↑ size of RBC's; folate or B12 deficiency |
|
|
Term
|
Definition
B12 deficiency; accumulation of methyl-THF = methyl trap |
|
|
Term
|
Definition
1) Methionine, 2) thymine, 3) purines |
|
|
Term
Glycine cleavage enzyme reaction |
|
Definition
Gly + THF → methylene-THF + HCO3 + NH4 |
|
|
Term
|
Definition
Met → SAM → SAH → homocysteine → Cys |
|
|
Term
|
Definition
|
|
Term
Methionine synthase cofactor |
|
Definition
|
|
Term
Methionine synthase uses: NADPH or NADP or NADH or NAD |
|
Definition
|
|
Term
Reasons to love phenylalanine (2) |
|
Definition
1) Essential, 2) in aspartame |
|
|
Term
Reasons to love tyrosine (4) |
|
Definition
Makes 1) melanin, 2) thyroglobin, 3) catecholamines, 4) DOPA |
|
|
Term
Catecholamine examples (3) |
|
Definition
1) Dopamine, 2) EPI, 3) NE |
|
|
Term
|
Definition
Molecular oxygen: 1 to substrate, 1 to make water |
|
|
Term
Phenylalanine hydroxylase reaction |
|
Definition
|
|
Term
|
Definition
DHB (quinoid) + NADH → THB + NAD |
|
|
Term
Active site of phenylalanine hydroxylase |
|
Definition
|
|
Term
DHB reductase: NADH or NAD or NADPH or NADP |
|
Definition
|
|
Term
DHF reductase: NADH or NAD or NADPH or NADP |
|
Definition
|
|
Term
Biopterin synthesis reactions |
|
Definition
|
|
Term
Phenylalanine deficiencies (2) |
|
Definition
1) Phenylalanine hydroxylase, or 2) THB synthesis |
|
|
Term
|
Definition
DHB (non-quinoid) + NADPH → THB + NADP |
|
|
Term
DHB → THB: which converts quinoid form to THB: DHB reductase or DHF reductase |
|
Definition
|
|
Term
DHB → THB: which converts non-quinoid form to THB: DHB reductase or DHF reductase |
|
Definition
|
|
Term
Synthesis of biopterin from what substrate |
|
Definition
|
|
Term
Catecholamine synthesis pathway (5 substrates starting from Tyr) |
|
Definition
Tyr → DOPA → dopamine → NE → EPI |
|
|
Term
Tyrosine hydroxylase reaction |
|
Definition
Tyr + O2 + THB → DOPA + H2O + DHB |
|
|
Term
DOPA → dopamine requires what cofactor |
|
Definition
|
|
Term
Dopamine → NE requires what "cofactors" (2) |
|
Definition
1) Cu, 2) Vitamin C (ascorbic acid) |
|
|
Term
NE → EPI requires what "cofactor" |
|
Definition
|
|
Term
Which reaction forms catechol group |
|
Definition
Tyrosine hydroxylase: Tyr → DOPA |
|
|
Term
|
Definition
Abnormal adregnergics: hypertension & cardiac problems; hih VMA in urine |
|
|
Term
Parkinson's treatment (4) |
|
Definition
1) MAOI, 2) tolcapone (COMT inhibitor, 3) entacapone (COMT inhibitor), 4) D2 agonists |
|
|
Term
Tissues that produce heme (2; most to least) |
|
Definition
1) Erythropoetic tissues (300 mg/day), 2) liver (50 mg/day) |
|
|
Term
Erythropoetic tissues mainly make what heme product |
|
Definition
|
|
Term
Liver mainly makes what heme product [not bilirubin] |
|
Definition
|
|
Term
Heme from __ SCoA + __ Glycines |
|
Definition
|
|
Term
|
Definition
SCoA + Gly → δ-aminolevulinic acid (ALA) + CoA |
|
|
Term
ALA synthase inhibited by |
|
Definition
|
|
Term
What stimulates globin production in RBC's |
|
Definition
|
|
Term
|
Definition
|
|
Term
ALA synthase requires what cofactor |
|
Definition
|
|
Term
|
Definition
|
|
Term
ALA dehyratase inhibited by |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Inner mitochondrial membrane |
|
|
Term
Erythropoietic protoporphyria |
|
Definition
PP-IX → free radicals in skin → skin damage; exteme sunlight sensitivity; absent heme synthesis feedback |
|
|
Term
Increasing cytochrome P450 synthesis: up- or downregulate heme synthesis |
|
Definition
|
|
Term
Neonatal jaundice caused by |
|
Definition
Late development of bilirubin conjugase |
|
|
Term
|
Definition
Problems in heme synthesis |
|
|
Term
Acute intermittent porphyria |
|
Definition
Abdominal & neurological problems; precipitated by P450 stimulating drugs |
|
|
Term
Degradation steps (4; starting with heme) |
|
Definition
Heme → biliverdin → indirect bilirubin → direct bilirubin |
|
|
Term
|
Definition
1) Neonatal, 2) hemolytic, 3) hepatocellular, 4) obstructive, 5) Crigler-Najjar |
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Term
Crigler-Najjar: which type is fatal |
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Definition
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Term
Crigler-Najjar: which type is not fatal |
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Definition
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Term
Jaundices with an increased indirect bilirubin |
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Definition
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Term
Jaundices with an increased direct bilirubin |
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Definition
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Term
Jaundice caused by cirrhosis |
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Definition
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Term
Jaundice caused by RBC lysis |
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Definition
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Term
Jaundice caused by decreased bilirubin conjugase |
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Definition
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Term
Jaundice caused deficiency of glucuronyltransferase |
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Definition
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Term
What binds to free heme and takes it to the spleen |
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Definition
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Term
Bilirubin pathway (4; starting with bilirubin) |
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Definition
Bilirubin → urobilinogen → urobilin → sterobilin |
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Term
Which bilirubin product is yellow |
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Definition
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Term
Which bilirubin product is brown |
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Definition
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Term
Indirect bilirubin: conjugated or unconjugated bilirubin |
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Definition
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Term
Direct bilirubin: conjugated or unconjugated bilirubin |
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Definition
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Term
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Definition
Indirect + direct bilirubin |
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Term
Vitamin K epoxide form: active or inactive |
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Definition
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Term
Vitamin K dependent carboxylase reaction |
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Definition
K + Glu residue + CO2 → K-epoxide + γ-carboxyglutamic acid residue |
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Term
Vitamin K reduction inhibited by |
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Definition
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Term
Vitamin K reduction can use which: NADPH or NADH |
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Definition
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