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Thiamine pyrophosphate: an essential cofactor in the mammalian metabolism of 3-methyl-branched fatty acids--implications for thiamine deficiencies?

作者信息

Foulon Veerle, Casteels Minne, Mannaerts Guy P, Gelb Bruce D, Van Veldhoven Paul P

机构信息

Afdeling Farmacologie, Departement Moleculaire Celbiologie, Katholieke Universiteit Leuven, Campus Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.

出版信息

Adv Exp Med Biol. 2003;544:305-6. doi: 10.1007/978-1-4419-9072-3_39.

DOI:10.1007/978-1-4419-9072-3_39
PMID:14713245
Abstract
摘要

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Thiamine pyrophosphate: an essential cofactor in the mammalian metabolism of 3-methyl-branched fatty acids--implications for thiamine deficiencies?焦磷酸硫胺素：哺乳动物3-甲基支链脂肪酸代谢中的必需辅因子——对硫胺素缺乏的影响？
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Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.2-羟基植烷酰辅酶A裂解酶的纯化、分子克隆及表达，该酶是一种过氧化物酶体中依赖硫胺焦磷酸的酶，在3-甲基支链脂肪酸的α-氧化过程中催化碳-碳键的裂解。
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Hepatic metabolism and transport in thiamine deficiency.硫胺素缺乏时的肝脏代谢与转运
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Thiamine pyrophosphokinase deficiency causes a Leigh Disease like phenotype in a sibling pair: identification through whole exome sequencing and management strategies.硫胺素焦磷酸激酶缺乏症在一对同胞中导致类 Leigh 病表型：通过全外显子组测序进行鉴定及管理策略
Mol Genet Metab Rep. 2014 Feb 11;1:66-70. doi: 10.1016/j.ymgmr.2013.12.007. eCollection 2014.