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8 Cards in this Set
- Front
- Back
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The reactions catalyzed by pyruvate dehydrogenase complex PDHC are analogous to those catalyzed by what enzyme?
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Alpha-Ketoglutarate dehydrogenase complex
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When PDH is phosphorylated is it active or inactive?
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Phosphorylated inactive and dephosphorylated active form
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What enzyme does arsenic inhibit? Production of what molecule is effected?
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Pyruvate Dehydrogenase
Acetly CoA |
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What cofactors are required by PDHC?
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Thiamine TPP)
Lipoic acid Pantothenate (FAD) Niacin (NAD) |
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What is the only membrane-bound enzyme of TCA?
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Succinate Dehydrogenase Complex II
Also enzyme of Oxidative Phosphorylation |
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If a patinet has a pyruvate carboxylase deficiency what metabolite is the pt unable to produce effectively?
What cofactor requird? Where enzyme located? |
Oxaloacetate
Pyruvate carboxylase catalyzes rxn and requires BIOTIN PC found in liver, brain, and adipose (but not muscle) Pyruvate>Oxaloacetate |
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If a patient presents with MELAS mitochondrial ENCEPHALOPATHY, lactic acidosis and STROKE-LIKE EPISODES, muscle weakness, vomiting, what is likely mtDNA mutation causing disease?
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80-90% of mutations are in tRNA
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Pt with mtDNA disorder (Leber's hereditary optic neuropathy (LHON)) have defective genes for what enzyme?
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NADH Dehydrogenase COMPLEX I
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