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

F Takada, DLV Woude, HQ Tong… - Proceedings of the …, 2001 - National Acad Sciences
F Takada, DLV Woude, HQ Tong, TG Thompson, SC Watkins, LM Kunkel, AH Beggs
Proceedings of the National Academy of Sciences, 2001National Acad Sciences
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 …
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.
National Acad Sciences