Author/Editor		Baker, Jane; Riley, Genna; Romero, M. Rosario; Haynes, Andrew R.; Hilton, Helen; Simon, Michelle M; Hancock, John M.; Tateossian, Hilda; Ripoll, Vera M.; Blanco, Gonzalo
Title		Identification of a Z-band associated protein complex involving KY, FLNC and IGFN1
Type		članek
Vol. and No.		Letnik 316, št. 11
Publication year		2010
Volume		str. 1856-1870
ISSN		0014-4827 - Experimental cell research
Language		eng
Abstract		The KY protein underlies a form of muscular dystrophy in the mouse but its role in muscle remains elusive. Immunodetection of endogenous KY protein in C2C12-derived myotubes and expression of a recombinant form in neonatal cardiomyocytes indicated that KY is a Z-band associated protein. Moreover, characterization of a KY interacting protein fragment led to the identification of Igfn1 (Immunoglobulin-like and fibronectin type 3 domain containing 1). Igfn1 is a transcriptionally complex locus encoding many protein variants. A yeast two-hybrid screen identified the Z-band protein filamin C (FLNC) as an interacting partner. Consistent with this, expression of an IGFN1 recombinant fragment showed that the three N-terminal globular domains, common to at least five IGFN1 variants, are sufficient to provide Z-band targeting. Taken together, the yeast two-hybrid, biochemical and immunofluorescence data support the notion that KY, IGFN1 and FLNC are part of a Z-band associated protein complex likely to provide structural support to the skeletal muscle sarcomere.
Keywords		muscular dystrophy cytoskeleton filamin C mišična distrofija filamin C