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Pyruvate carboxylase deficiency—insights from liver transplantation

✍ Scribed by William L Nyhan; Ajai Khanna; Bruce A Barshop; Robert K Naviaux; Andrew F Precht; Joel E Lavine; Marquis A Hart; Bryan E Hainline; Rebecca S Wappner; Sharon Nichols; Richard H Haas

Book ID
117735415
Publisher
Elsevier Science
Year
2002
Tongue
English
Weight
109 KB
Volume
77
Category
Article
ISSN
1096-7192

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Pyruvate carboxylase (PC), a key enzyme for gluconeogenesis and anaplerotic pathways, consists of four domains, namely, biotin carboxylase (BC), carboxyltransferase (CT), pyruvate carboxylase tetramerization (PT), and biotin carboxyl carrier protein (BCCP). PC deficiency is a rare metabolic disorder