Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice.

Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice. Research Abstract Details 

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Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice. Abstract Text:

Zhi-Fang Li,Xiaohua Wu,Yun Jiang,Jianxiang Liu,Chun Wu,Masaki Inagaki,Ichiro Izawa,Andrew P Mizisin,Eva Engvall,G Diane Shelton,

Protein aggregate formation in muscle is thought to be pathogenic and associated with clinical weakness. Over-expression of either wild type or a mutant form of myeloid leukemia factor 1 (MLF1) in transgenic mouse skeletal muscle and in cultured cells resulted in aggregate formation. Aggregates were detected in MLF1 transgenic mice at 6 weeks of age, and increased in size with age. However, histological examination of skeletal muscles of MLF1 transgenic mice revealed no pathological changes other than the aggregates, and RotaRod testing did not detect functional deficits. MLF1 has recently been identified as a protein that could neutralize the toxicity of intracellular protein aggregates in a Drosophila model of Huntington's disease (HD). We also demonstrate that MLF1 interacts with MRJ, a heat shock protein, which can independently neutralize the toxicity of intracellular protein aggregates in the Drosophila HD model. Our data suggest that over-expression of MLF1 has no significant impact on skeletal muscle function in mice; that progressive formation of protein aggregates in muscle are not necessarily pathogenic; and that MLF1 and MRJ may function together to ameliorate the toxic effects of polyglutamine or mutant proteins in myodegenerative diseases such as inclusion body myositis and oculopharyngeal muscular dystrophy, as well as neurodegenerative disease.

Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice. Publishing Authors By Initials

ZF Li,X Wu,Y Jiang,J Liu,C Wu,M Inagaki,I Izawa,AP Mizisin,E Engvall,GD Shelton,

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Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Journal of the neurological sciences

VOLUME: 264

Page Numbers: 77-86

Journal Abbreviation: J. Neurol. Sci.

ISSN: 0022-510X

DAY: 12

MONTH: 09

YEAR: 2007

Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice. Information

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LANGUAGE: eng

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Grant and Affiliation Information for Non-pathogenic protein aggregates in skeletal muscle in MLF1 transgenic mice.

AFFILIATION: Burnham Institute for Medical Research, 10901 N. Torrey Pines Road. La Jolla, CA 92037, United States.

Country: Netherlands

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MEDLINETA: J Neurol Sci

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