PDHB (Pyruvate dehydrogenase E1 component subunit beta, mitochondrial) is also named as PHE1B. The enzyme, which is found in mitochondria, is one of the component enzymes of the pyruvate dehydrogenase multienzyme complex (PDH). It catalyzes the first reaction of an oxidative decarboxylation sequence converting pyruvate to acetyl-CoA and CO(2). Defects in PDHB are the cause of pyruvate dehydrogenase E1-beta deficiency (PDHBD).
蛋白别名: PDHE1-B; Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; pyruvate dehydrogenase, E1 beta polypeptide
基因别名: 2610103L06Rik; AL024199; C81408; PDHB; PDHBD; PDHE1-B; PHE1B