【用戶】涵涵（邀請碼107252）

【年級】

【評論內容】Pyruvate oxidation to acetyl-CoA is catalyzed by the pyruvate dehydrogenase complex (PDH complex). It is an oxidative decarboxylation, which is virtually irreversible involving three enzymes and five coenzymes. Thiamine pyrophosphate (TPP), one of the cofactor in pyruvate dehydrogenase, participates in decarboxylation of pyruvate, yielding hydroxyethyl-TPP for next step. In clinical study, patients with PDH complex deficiency elevate plasma pyruvate levels.The pyruvate carboxylase reaction requires the vitamin biotin, which is the prosthetic group of the enzyme. But lacking biotin or pyruvate carboxylate does not cause high level of pyruvate in their blood.Reference: Pyruvate dehydrogenase complex deficiency: updating the clinical, metabolic and mutational landscapes in a cohort of Portuguese patients. Orphanet J Rare Dis (2020) 15:298維持原公布答案(C)

【用戶】涵涵（邀請碼107252）

【年級】

【評論內容】Pyruvate oxidation to acetyl-CoA is catalyzed by the pyruvate dehydrogenase complex (PDH complex). It is an oxidative decarboxylation, which is virtually irreversible involving three enzymes and five coenzymes. Thiamine pyrophosphate (TPP), one of the cofactor in pyruvate dehydrogenase, participates in decarboxylation of pyruvate, yielding hydroxyethyl-TPP for next step. In clinical study, patients with PDH complex deficiency elevate plasma pyruvate levels.The pyruvate carboxylase reaction requires the vitamin biotin, which is the prosthetic group of the enzyme. But lacking biotin or pyruvate carboxylate does not cause high level of pyruvate in their blood.Reference: Pyruvate dehydrogenase complex deficiency: updating the clinical, metabolic and mutational landscapes in a cohort of Portuguese patients. Orphanet J Rare Dis (2020) 15:298維持原公布答案(C)