Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia

Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia

Background: Citrin, encoded by SLC25A13, is a component of the malate-aspartate shuttle, which is the main NADH-transporting system in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD), which usually resolves within the first year of life. However, small numbers of adults...

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Bibliographic Details
Main Authors: Kiyoshi Hayasaka, Chikahiko Numakura, Kentaro Toyota, Satoru Kakizaki, Hisayoshi Watanabe, Hiroaki Haga, Hiroshi Takahashi, Yoshimi Takahashi, Mieko Kaneko, Mitsunori Yamakawa, Hiroyuki Nunoi, Takeo Kato, Yoshiyuki Ueno, Masatomo Mori
Format: Article
Language:English
Published: Elsevier 2014-01-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Neonatal intrahepatic cholestasis (NICCD)
Adult-onset type II citrullinemia (CTLN2)
Citrin deficiency
Medium-chain triglycerides (MCT)
SLC25A13
Malate-aspartate shuttle
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426913000074

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