Journal
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Volume 98, Issue 4, Pages 1595-1600Publisher
NATL ACAD SCIENCES
DOI: 10.1073/pnas.041609698
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Funding
- NIAMS NIH HHS [K02 AR02026, R01 AR044345, R01 AR44345] Funding Source: Medline
- NICHD NIH HHS [P30 HD018655, P30 HD18655] Funding Source: Medline
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To better understand the structure and function of Z lines, we used sarcomeric isoforms of ol-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 cy-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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