HSA21基因GABPalpha的组织特异性过表达:对退行性痴呆的影响。

IF 4.2 2区生物学 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY

Biochimica et biophysica acta. Molecular basis of disease Pub Date : 2004-12-24 DOI:10.1016/j.bbadis.2004.09.005

Debra A. O'Leary, Melanie A. Pritchard, Dakang Xu, Ismail Kola , Paul J. Hertzog, Sika Ristevski

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引用次数: 0

摘要

ETS转录因子GABPalpha由HSA21上的一个基因编码，并与含有锚蛋白重复序列的β亚基相互作用形成GABP复合物。GABP调节参与线粒体呼吸和神经肌肉信号传导的基因表达。当GABPalpha mRNA在人DS成纤维细胞系中过表达或转染NIH3T3细胞时，未检测到蛋白水平升高。然而，在Ts65Dn片段三体小鼠DS模型(DS)中，Gabpalpha基因剂量的增加只导致Gabpalpha蛋白在脑和骨骼肌中的水平升高。这些发现提示GABPalpha蛋白水平以组织特异性的方式受到严格调节，因此GABP可能在GABPalpha蛋白水平升高的组织中发挥作用。

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Tissue-specific overexpression of the HSA21 gene GABPα: implications for DS

The ETS transcription factor GABPα is encoded by a gene on HSA21 and interacts with an ankyrin repeat-containing β subunit to form the GABP complex. GABP regulates expression of genes involved in mitochondrial respiration and neuromuscular signalling. When GABPα mRNA is overexpressed in human DS fibroblast cell lines, or by tranfection in NIH3T3 cells, no increase in protein level is detected. However, increased Gabpα gene dosage in the Ts65Dn segmental trisomy mouse model of DS (DS) results in elevated Gabpα protein levels in brain and skeletal muscle only. These findings suggest that GABPα protein levels are tightly regulated in a tissue-specific manner, and consequently GABP may play a role in DS pathologies in tissues where GABPα protein levels are elevated.

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来源期刊

Biochimica et biophysica acta. Molecular basis of disease 生物-生化与分子生物学

CiteScore

12.30

自引率

0.00%

发文量

218

审稿时长

32 days

期刊介绍： BBA Molecular Basis of Disease addresses the biochemistry and molecular genetics of disease processes and models of human disease. This journal covers aspects of aging, cancer, metabolic-, neurological-, and immunological-based disease. Manuscripts focused on using animal models to elucidate biochemical and mechanistic insight in each of these conditions, are particularly encouraged. Manuscripts should emphasize the underlying mechanisms of disease pathways and provide novel contributions to the understanding and/or treatment of these disorders. Highly descriptive and method development submissions may be declined without full review. The submission of uninvited reviews to BBA - Molecular Basis of Disease is strongly discouraged, and any such uninvited review should be accompanied by a coverletter outlining the compelling reasons why the review should be considered.