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

Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1595-600. doi: 10.1073/pnas.98.4.1595. Epub 2001 Feb 6.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

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

Substances

  • Carrier Proteins
  • Contractile Proteins
  • DNA, Complementary
  • Filamins
  • MYOZ1 protein, human
  • Microfilament Proteins
  • Muscle Proteins
  • Actinin

Associated data

  • GENBANK/AF240633
  • GENBANK/AF243386
  • GENBANK/AF243387
  • GENBANK/AF243388
  • GENBANK/AF243389
  • GENBANK/AF243390