Subcellular distribution and cofactor function of human branched chain α-ketoacid dehydrogenase in normal and mutant cultured skin fibroblasts-Reference-Cited by-同舟云学术

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Subcellular distribution and cofactor function of human branched chain α-ketoacid dehydrogenase in normal and mutant cultured skin fibroblasts

Published:1975-05 Issue:1 Volume:13 Page:7-22
ISSN:0006-2944
Container-title:Biochemical Medicine
language:en
Short-container-title:Biochemical Medicine

Author:

Danner Dean J.,Elsas Louis J.

Publisher

Elsevier BV

Subject

Biochemistry

Reference32 articles.

1. "Maple Syrup Urine Disease"

2. Amino Acid Metabolism and Genetic Variation;Dancis,1967

3. Intermittent Branched-Chain Ketonuria

Cited by 24 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. A distinct variant of intermediate maple syrup urine disease;Clinical Genetics;2008-04-23

2. Molecular defects of the branched-chain α-keto acid dehydrogenase complex: Maple syrup urine disease due to mutations of the E1α or E1β subunit gene;Alpha-Keto Acid Dehydrogenase Complexes;1996

3. Transamination and Oxidative Decarboxylation Rates of Branched-Chain 2-Oxo Acids in Cultured Human Skin Fibroblasts;Pediatric Research;1988-01

4. [18] Enzyme assays with mutant cell lines of maple syrup urine disease;Methods in Enzymology;1988

5. Altered kinetic properties of the branched-chain alpha-keto acid dehydrogenase complex due to mutation of the beta-subunit of the branched-chain alpha-keto acid decarboxylase (E1) component in lymphoblastoid cells derived from patients with maple syrup urine disease.;Journal of Clinical Investigation;1987-07-01

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