Myozenin:: An α-actinin- and γ-filamin-binding protein of skeletal muscle Z lines

被引:173
作者
Takada, F
Vander Woude, DL
Tong, HQ
Thompson, TG
Watkins, SC
Kunkel, LM
Beggs, AH
机构
[1] Childrens Hosp, Div Genet, Boston, MA 02115 USA
[2] Harvard Univ, Sch Med, Dept Pediat, Boston, MA 02115 USA
[3] Howard Hughes Med Inst, Boston, MA 02115 USA
[4] Univ Pittsburgh, Sch Med, Dept Physiol & Cell Biol, Pittsburgh, PA 15261 USA
关键词
D O I
10.1073/pnas.041609698
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
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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页码:1595 / 1600
页数:6
相关论文
共 38 条
  • [1] SYNTROPHIN BINDS TO AN ALTERNATIVELY SPLICED EXON OF DYSTROPHIN
    AHN, AH
    KUNKEL, LM
    [J]. JOURNAL OF CELL BIOLOGY, 1995, 128 (03) : 363 - 371
  • [2] BEGGS AH, 1992, J BIOL CHEM, V267, P9281
  • [3] CELL TYPE-SPECIFIC EXPRESSION OF JC-VIRUS T-ANTIGEN IN PRIMARY AND ESTABLISHED CELL-LINES FROM TRANSGENIC MICE
    BEGGS, AH
    MINER, JH
    SCANGOS, GA
    [J]. JOURNAL OF GENERAL VIROLOGY, 1990, 71 : 151 - 164
  • [4] THE STRUCTURE AND FUNCTION OF ALPHA-ACTININ
    BLANCHARD, A
    OHANIAN, V
    CRITCHLEY, D
    [J]. JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, 1989, 10 (04) : 280 - 289
  • [5] In vitro expressed dystrophin fragments do not associate with each other
    Chan, YM
    Kunkel, LM
    [J]. FEBS LETTERS, 1997, 410 (2-3) : 153 - 159
  • [6] Human skeletal muscle-specific α-actinin-2 and -3 isoforms form homodimers and heterodimers in vitro and in vivo
    Chan, YM
    Tong, HQ
    Beggs, AH
    Kunkel, LM
    [J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1998, 248 (01) : 134 - 139
  • [7] THE 2-HYBRID SYSTEM - A METHOD TO IDENTIFY AND CLONE GENES FOR PROTEINS THAT INTERACT WITH A PROTEIN OF INTEREST
    CHIEN, CT
    BARTEL, PL
    STERNGLANZ, R
    FIELDS, S
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1991, 88 (21) : 9578 - 9582
  • [8] ZASP: A new Z-band alternatively spliced PDZ-motif protein
    Faulkner, G
    Pallavicini, A
    Formentin, E
    Comelli, A
    Ievolella, C
    Trevisan, S
    Bortoletto, G
    Scannapieco, P
    Salamon, M
    Mouly, V
    Valle, G
    Lanfranchi, G
    [J]. JOURNAL OF CELL BIOLOGY, 1999, 146 (02) : 465 - 475
  • [9] FATZ, a filamin-, actinin-, and telethonin-binding protein of the Z-disc of skeletal muscle
    Faulkner, G
    Pallavicini, A
    Comelli, A
    Salamon, M
    Bortoletto, G
    Ievolella, C
    Trevisan, S
    Kojic, S
    Dalla Vecchia, F
    Laveder, P
    Valle, G
    Lanfranchi, G
    [J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 2000, 275 (52) : 41234 - 41242
  • [10] Calsarcins, a novel family of sarcomeric calcineurin-binding proteins
    Frey, N
    Richardson, JA
    Olson, EN
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (26) : 14632 - 14637