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23 Cards in this Set

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oxaloacetate transaminated-->
aspartate
aspartate transaminated -->
oxaloacetate
glucokinase vs hexokinase
(both key regulatory enzymes in glycolysis); gluc--only in liver, high Km (low affinity)--> v active after meal, induced when insulin high; hex--in ~all tissues, low Km (high affinity) so works even when [glucose ] low. feedback inhib by G6P
regulators of PFK-1
(rate limiting step in glycolysis); neg--ATP, citrate; pos--AMP, F2,6BP
regulators of pyruvate kinase
(PEP->pyruvate); neg--alanine, ATP; pos--F1,6BP
None
regulators of pyruvate DH
(pyruvate->acetyl CoA); neg--ATP, NADH, acetyl CoA
why can alcohol cause hypoglycemia?
metabolism of alchohol causes increased NADH...which inhibits gluconeogenesis
thiamine deficiency effect on TCA cycle?
thiamine needed for pyruvate DH and alpha KG DH. therefore, TCA slows, get lactic acidosis
why are glycolytic enzyme deficiencies assoc'd with hemolytic anemia?
RBCs can only metabolize glucose anaerobically. so if def--> no ATP
cofactors needed for pyruvate DH (and alpha KG DH)
pyrophosphate (B1, TPP); FAD (B2); NAD (B3); CoA (B5); lipoic acid
significance of As?
inhibits lipoic acid (cofactor for pyruvate DH, alpha KG DH); get vomiting, rice water poo, garlic breath
significance of Cori cycle
transfers excess reducing equivalents from RBCs/mm to liver, allowing muscle to function anaerobically and shifting net metabolic burden to liver; net 2ATP.
treatment of pyruvate DH def?
increase intake of KETOgenic amino acids (eat a lot of fat and/or lotsa lysine, leucine)
which stress hormones promote gluconeogenesis
glucagon, epinephrine, GH, glucocorticoids
where is glucose 6 phosphatase?
(G6P->G); in ER of liver, kidney, intestinal epithelium
where are the key regulatory (irreversible) enzymes of gluconeogenesis (4)
pyruvate carboxylase-in mit; PEP CK--cyt; F1,6BPase--cyt; G6Pase--cyt
specific requirements of irreversible gluconeogenesis enyzmes
PC--ATP, biotin; PEP CK--GTP
products of nonoxidative/reversible reactio of PPP/HMP shunt
ribose 5-phophsate (for NT); G3P, F6P (glycolytic intermediates)
absence of galactose-1-phosphate uridyltransferase-->
(AR) cataracts, hepatosplenomegaly, MR; due to accumulation of toxic substances (galactitol)
rate limiting step in urea cycle?
carbamoyl phosphate synthase I (CO2+ NH4+--> carbamoyl phosphate)
pyruvate transaminated-->
alanine
forms of homocystinuria
(all AR); 1. cystathione synthase def; 2. dec'd affinity of cystathione synthase for pyridoxal pohsphate (tx: inc. vit B6); 3. methionine synthase def
homocystinuria can cause
MR (mental retardation), osteoporosis, tall stature, kyphosis, lens subluxation, atherosclerosis