Myozenin: An α-actinin- and γ-Filamin-Binding Protein of Skeletal Muscle Z Lines

To better understand the structure and function of Z lines, we used sarcomeric isoforms of α-actinin and γ-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 α-actinin- and γ-filamin-binding Z lin...

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Bibliographic Details
Published in:Proceedings of the National Academy of Sciences - PNAS 2001-02, Vol.98 (4), p.1595-1600
Main Authors: Takada, Fumio, Douglas L. Vander Woude, Tong, Hui-Qi, Thompson, Terri G., Watkins, Simon C., Kunkel, Louis M., Beggs, Alan H.
Format: Article
Language:English
Subjects:
Actinin - metabolism
Amino Acid Sequence
Amino acids
Base Sequence
Binding Sites
Binding, Competitive
Biological Sciences
Carrier Proteins - genetics
Carrier Proteins - metabolism
chromosome 10
Cloning, Molecular
Contractile Proteins - metabolism
DNA, Complementary
Exons
Filamins
g-filamin-binding protein
Genes
Genomics
Humans
Microfilament Proteins - metabolism
Microscopy
Molecular Sequence Data
Muscle Proteins - genetics
Muscle Proteins - metabolism
Muscle, Skeletal - metabolism
MYOZ gene
myozenin
Proteins
Skeletal muscle
Transmission lines
Yeasts
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Summary:To better understand the structure and function of Z lines, we used sarcomeric isoforms of α-actinin and γ-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 α-actinin- and γ-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 α-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 α-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.
ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.041609698