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  • Myozenin: an alpha-actinin- and gamma-filamin-binding protein of skeletal muscle Z lines.

Myozenin: an alpha-actinin- and gamma-filamin-binding protein of skeletal muscle Z lines.

Proceedings of the National Academy of Sciences of the United States of America (2001-02-15)
F Takada, D L Vander Woude, H Q Tong, T G Thompson, S C Watkins, L M Kunkel, A H Beggs
摘要

To better understand the structure and function of Z lines, we used sarcomeric isoforms of alpha-actinin and gamma-filamin to screen a human skeletal muscle cDNA library for interacting proteins by using the yeast two-hybrid system. Here we describe myozenin (MYOZ), an alpha-actinin- and gamma-filamin-binding Z line protein expressed predominantly in skeletal muscle. Myozenin is predicted to be a 32-kDa, globular protein with a central glycine-rich domain flanked by alpha-helical regions with no strong homologies to any known genes. The MYOZ gene has six exons and maps to human chromosome 10q22.1-q22.2. Northern blot analysis demonstrated that this transcript is expressed primarily in skeletal muscle with significantly lower levels of expression in several other tissues. Antimyozenin antisera stain skeletal muscle in a sarcomeric pattern indistinguishable from that seen by using antibodies for alpha-actinin, and immunogold electron microscopy confirms localization specifically to Z lines. Thus, myozenin is a skeletal muscle Z line protein that may be a good candidate gene for limb-girdle muscular dystrophy or other neuromuscular disorders.

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单克隆抗肌球蛋白(骨骼肌快肌) 小鼠抗, clone MY-32, ascites fluid