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

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.

Pubmed ID: 11171996

Authors

  • Takada F
  • Vander Woude DL
  • Tong HQ
  • Thompson TG
  • Watkins SC
  • Kunkel LM
  • Beggs AH

Journal

Proceedings of the National Academy of Sciences of the United States of America

Publication Data

February 13, 2001

Associated Grants

  • Agency: NIAMS NIH HHS, Id: K02 AR02026
  • Agency: NICHD NIH HHS, Id: P30 HD18655
  • Agency: NIAMS NIH HHS, Id: R01 AR44345

Mesh Terms

  • Actinin
  • Amino Acid Sequence
  • Base Sequence
  • Binding Sites
  • Binding, Competitive
  • Carrier Proteins
  • Cloning, Molecular
  • Contractile Proteins
  • DNA, Complementary
  • Filamins
  • Humans
  • Microfilament Proteins
  • Molecular Sequence Data
  • Muscle Proteins
  • Muscle, Skeletal