Oxidative energy deficiency. II. Human achondroplasia

Oxidative energy deficiency. II. Human achondroplasia
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氧化能缺乏。

DOI:
10.1016/0003-9861(73)90531-6
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发表时间:
1973
影响因子:
3.9
通讯作者:
M. Cohen
M. Cohen
中科院分区:
生物学3区
文献类型:
--
作者:
B. Mackler;B. Haynes;A. R. Inamdar;L. R. Pedegana;J. Hall;M. Cohen

文献摘要

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从典型软骨发育不全受试者的肌肉活检标本中分离的线粒体制备物已证明存在氧化能形成缺陷(可能在磷酸化位点I(终末呼吸系统的NADH脱氢酶区域)磷酸化降低)。
Defective oxidative energy formation (with decreased phosphorylation possibly occurring at Site I of phosphorylation, the NADH dehydrogenase region of the terminal respiratory system) has been demonstrated in mitochondrial preparations isolated from muscle biopsy specimens from human subjects with classical achondroplasia.