Term
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Definition
| Forms bonds with Fe2+ on alpha helix |
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Term
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Definition
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Term
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Definition
| Major adult Hb (glu on B chain) |
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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
| Nonenzymatically glycosylated alpha and beta chains at N'; marker of diabetes mellitus, hyperglycemia |
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Term
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Definition
| Reduces O2 affinity therefore is useless as an O2 transporter; excessive methemaglobin formation |
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Term
| RBCs defend against oxidation of heme Fe2+ via: |
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Definition
| Ascorbate (Vitamin C), glutathione; proper binding of heme creating protective environment; methemoglobin reductase |
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Term
| Ascorbate (vit. C), glutathione |
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Definition
| React with oxidative agents before they can react with and care damage to Hb Fe2+ |
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Term
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Definition
| Reduces methemaglobin to normal hemoglobin; requires NADH coenzyme (Fe3+ + NADH, H --> Fe2+ + NAD+) |
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Term
| Congenital methemoglobinemia (HbM) |
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Definition
| Abnormality in heme binding pocket of alpha or beta chain due to substitution of proximal histadine by tyrosine |
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Term
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Definition
| Binds O2 150-300x more tightly than does T conformation; O2 binding pulls proximal histadine causes T-->R conformation change |
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Term
| Hb cooperativity and sigmoidal kinetics |
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Definition
| As pO2 rises, heme #1 is oxygenated --> Hb shifts to R state with higher O2 affinity which allows binding of heme #2-4 |
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Term
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Definition
| Negative effector of O2 binding to Hb; promotes unloading of O2 to tissues; rapidly rises in hypoxia; allows O2 unloading from HbA to HbF |
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Term
| Binding of BPG to deoxyhemoglobin ("T") |
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Definition
| Occurs via 8x histadyl groups ONLY on the beta chains |
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Term
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Definition
| CO2 covalently binds terminal -NH2 of both alpha and beta chains |
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Term
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Definition
| Major facilitator of O2 unloading in tissues |
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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
| Henderson-Hasselbalch equation |
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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
| Buffer parallels H2O's temperature dependent behavior |
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Term
| Chloride-bicarbonate exchanger (anion exchange protein) |
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Definition
| Increases membrane permeability >10^6; secondary active transport |
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Term
| Transmembrane transporters ("cassettes") |
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Definition
| Na/K ATPase, Ca2+ transporter, CFTCR (cyctic fibrosis transmembrane conductance regulator = Cl- transporter) |
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Term
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Definition
| e- transfer via oxidation-reduction reaction |
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Term
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Definition
| Transfer of functional groups to a substrate |
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Term
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Definition
| Donor group transferred to water; catalyzes cleavage of bonds by addition of water |
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Term
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Definition
| Addition of groups to double bonds/formation of double bonds by group removal; adds/removes water, ammonia, CO2 |
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Term
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Definition
| Transfers group within a molecule; catalyzes racemization of geometric/optical isomers; chemical formula remains the same, freely reversable |
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Term
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Definition
| Formation of bonds between -C,O,N,S via condensation reactions coupled to ATP |
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Term
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Definition
| Number of substrate molecules --> products/sec |
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Term
| Transition metal cofactors |
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Definition
| Zn2+, Fe2/3+, Mn2+, Cu2+/++; promote catalysis by binding substrate and promoting electophilic catlaysis at bond cleavage site or stabilizing intermediates in reaction sequence |
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Term
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Definition
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Term
| Michaelis-Menten equation |
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Definition
| V = (Vmax * [S])/(Km + [S]) |
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Term
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Definition
| Increase in Km, no change in Vmax; statin, allopurinol |
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Term
| Non-competitive inhibition |
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Definition
| Decrease in Vmax, no change in Km |
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Term
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Definition
| Decrease in Km and Vmax; formation of permanent adduct (binds ES complex, not substrate binding site); organophosphates, aspirin, cholera toxin |
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Term
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Definition
| Permanently binds to ACh-ase |
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Term
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Definition
| Inhibits COX1, 2 via permanent binding to a serine residue at active site |
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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
| Covalent protein modification |
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Definition
| Cholera, Diptheria, Pertussis toxins |
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Term
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Definition
| Keeps adenylate cyclase 'on' via adenyl-ribosylation of key enzymes |
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Term
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Definition
| Enzyme inhibits protein synthesis by modification of a crutial histadine residue of eEf2; most resurgent disease |
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Term
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Definition
| ADP ribosylation of alpha subunit of G protein |
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Term
| Phosphorylation/dephosphorylation sites |
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Definition
| At reactive -OH groups on serine, threonine, tyrosine residues |
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Term
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Definition
| Specific for cardiac infarct |
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Term
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Definition
| More accurate and reliable than creatine kinase; peaks at 8-28 hours |
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Term
| Lactate dehydrogenase, glutamate-oxaloacetate transaminase |
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Definition
| Appear long after tissue damate (not as useful as infarct-specific indicators) |
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Term
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Definition
| Glucose cotransport driven by Na+ in intestinal epithelium, renal tubule |
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Term
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Definition
| Heart, brain, placenta, RBC; hypoxia in muscle = increased activity; also transports vitamin C |
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Term
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Definition
| Liver, kidney, pancreatic B cells, intestine; insulin independent |
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Term
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Definition
| Neurons, kidney, placenta; also transports vitamin C |
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Term
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Definition
| Muscle, apidocyte, heart; insulin dependent |
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Term
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Definition
| Transports fructose; located in sarcolemma (muscle) or vesicles (spermatozoon) |
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Term
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Definition
| Used at increased [glucose] due to increased Km |
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Term
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Definition
| Used at decreased [glucose] due to decreased Km |
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Term
| Phosphoglycerase kinase deficiency inheritance |
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Definition
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Term
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Definition
| Main regulatory enzymes of glycolysis |
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Term
| Pyruvate kinase deficiency |
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Definition
| Most common genetic defect causing hemolysis |
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Term
| Glycogenesis Type VII (Tarui's disease) |
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Definition
| PFK1 deficiency in skeletal muscle; inability to produce large amounts of lactose |
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Term
| Major precursors of glyconeogenesis |
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Definition
| Lactate, ketoacids (puruvate, OAA, alpha-KG derived from alanine, aspartate, glutamate), glycerol (from TAG), propionyl-CoA (from odd chain fatty acids) |
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Term
| von Gierke's disease (glycogenosis I) |
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Definition
| Deficiency in hepatic G6Pase leading to deep hypoglycemia and severe lactic acidosis ; uricemia leading to breakdown of puring nucleotides; xanthomata = increased serum lipids |
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Term
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Definition
| No direct role in buffering plasma glucose; muscle lacks G6Pase |
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Term
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Definition
| Only creates alpha-1,4 linkages always at the non-reducing end of a pre-existing chain |
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Term
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Definition
| Attaches initial glucose from UDP-glucose at the OH group of a specific tyrosine; autocatalytic; occurs 8x, then glycogen synthase takes over |
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Term
| Nucleoside diphosphate kinase |
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Definition
| In mitochondrial intermembrane space: UDP + ATP --> UTP + ADP |
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Term
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Definition
| Use of Pi to create G1P from G6P; covalently bound pyridoxal phosphate in Schiff base (connection between lysine and coenzyme also seen in biotin) |
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Term
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Definition
| C=N bound to lysyl residye |
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Term
| Debranching enzyme transferase activity |
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Definition
| Transfers alpha 1,4 attached glucose to a pre-existing chain |
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Term
| Debranching enzyme alpha-1,6 glucosidase activity |
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Definition
| Hydrolizes branchpoint glucose as free glucose |
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Term
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Definition
| Lysosomal degradation of glycogen granules to free glucose; cleaves both alpha1,4 and alpha1,6 linkages |
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Term
| Phosphoprotein phosphatases |
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Definition
| Recognize all seryl-phosphate groups |
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Term
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Definition
| Glycogen synthase deficiency; hepatic disorder; fasting hypoglycemia, ketosis, unresponsive to glucagon |
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Term
| Type IV glycogenoses (Anderson's disease) |
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Definition
| Branching enzyme deficiency; progressive hepato-splenomegaly (muscle also affected); long needle-like straight chains of alpha1-4 linkages |
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Term
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Definition
| Liver glycogen phosphorylase deficiency; X-linked; hepatomegaly |
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Term
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Definition
| Phosphorylase B kinase deficiency; involves muscle, liver |
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Term
| Type V glycogenoses (McArdle's syndrome) |
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Definition
| Muscle phosphorylase deficiency; effects skeletal muscle only; myoglobinuria, loss of creatine kinase --> renal failure |
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Term
| Type III glycogenoses (Cori disease) |
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Definition
| Debrancher deficiency; affects liver, muscle (hepatomegaly) |
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Term
| Type I glycogenoses (von Gierke's disease) |
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Definition
| G6Pase deficiency = insufficient free glucose; severe lactic acidosis; hepatomegaly; uricemia from excess purine breakdown (no conversion of lactate to glucose) |
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Term
| Type II glycogenoses (Pompe's disease) |
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Definition
| Acid alpha-glucosidase/acid maltase deficiency; lysosomal defect; muscle hypertrophy, CNS, liver affected; cardiomegaly, respiratory distress; floppy infant syndrome; no abnormalities in glycogen and energy metabolism |
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Term
| Type VII (Tarui's disease) |
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Definition
| PFK deficiency; elevated muscle glycogen; RBC hemolysis and hyperbilirubinemia |
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Term
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Definition
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Term
| Pyruvate dehydrogenase complex |
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Definition
| Inactivated with integral serine specific protein kinase (phosphorylated state); activated with integral phosphatase (dephosphorylated state); requires TPP |
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Term
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Definition
| TPP, FAD, NAD, CoASH, Lipoic acid (susceptable to arsenic poisoning) |
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Term
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Definition
| underog reversable oxidation/reduction |
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Term
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Definition
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Term
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Definition
| Driven by exogonic rupture of thioester bond |
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Term
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Definition
| Inhibited by Zn2+, fluroracetate (uncompetitive) |
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Term
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Definition
| Major regulator; irreversible; phosphorylation at critical serine prevents binding of isocitrate |
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Term
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Definition
| Requires CoASH; arsenite binds reactive -SH group |
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Term
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Definition
| Substrate level phosphorylation |
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Term
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Definition
| Membrane bound; powerfully inhibited by aresenite binding exposed -SH group of cysteinyl residues |
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Term
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Definition
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Term
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Definition
| In liver, released from mitochondria to inhibit PFK1; in heart, 100% is used for ATP production |
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