LOW-LEVELS OF MITOCHONDRIAL TRANSCRIPTION FACTOR-A IN MITOCHONDRIAL-DNA DEPLETION
LOW-LEVELS OF MITOCHONDRIAL TRANSCRIPTION FACTOR-A IN MITOCHONDRIAL-DNA DEPLETION
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DOI：
10.1006/bbrc.1994.1603
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发表时间：
1994-05-16
影响因子：
3.1
通讯作者：
CLAYTON, DA
中科院分区：
文献类型：
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作者：
LARSSON, NG;OLDFORS, A;CLAYTON, DA
低水平的线粒体转录因子A（mtTFA）被发现在肌纤维与线粒体DNA（mtDNA）耗尽在一个孩子致命的线粒体肌病，也在人类细胞系缺乏mtDNA。高水平的这种转录激活剂被发现在肌纤维与线粒体DNA的积累在两个病人与单一的mtDNA缺失。这是第一个核基因产物随人类mtDNA水平变化的例子。由于转录是mtDNA复制所必需的，因此mtTFA可能参与mtDNA复制的调控。(C)1994年出版社出版。
Low levels of mitochondrial transcription factor A (mtTFA) were found in muscle fibers with mitochondrial DNA (mtDNA) depletion in a child with fatal mitochondrial myopathy and also in a human cell line lacking mtDNA. High levels of this transcriptional activator were found in muscle fibers with mtDNA accumulation in two patients with single mtDNA deletions. This is the first example of a nuclear gene product that varies with the levels of mtDNA in humans. Since transcription is required for the replication of mtDNA, it is possible that mtTFA may be involved in the regulation of mtDNA replication. (C) 1994 Academic Press, Inc.