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Human Molecular Genetics Advance Access originally published online on September 22, 2004
Human Molecular Genetics 2004 13(22):2863-2874; doi:10.1093/hmg/ddh308
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Human Molecular Genetics, Vol. 13, No. 22 © Oxford University Press 2004; all rights reserved

Filamin C interacts with the muscular dystrophy KY protein and is abnormally distributed in mouse KY deficient muscle fibres

Jane Beatham1, Rosario Romero1, Stuart K.M. Townsend1, Terry Hacker1, Peter F.M. van der Ven2 and Gonzalo Blanco1,*

1MRC Mammalian Genetics Unit, Harwell, Oxon OX11 0RD, UK and 2Department of Cell Biology, University of Potsdam, Lennestr. 7a, D-14471 Potsdam, Germany

Received August 1, 2004; Accepted September 16, 2004

The KY protein has been implicated in a neuromuscular dystrophy in the mouse, but its role in muscle function remains unclear. Here, we show that KY interacts with several sarcomeric cytoskeletal proteins including, amongst others, filamin C and the slow isoform of the myosin-binding protein C. These interactions were confirmed in vitro and because of its central role in skeletal muscle disease, characterized in more detail for filamin C. A role for KY in regulating filamin C function in vivo is supported by the expression analysis of filamin C in the null ky mouse mutant, where distinct irregular subcellular localization of filamin C was found in subsets of muscle fibres, which appears to be a specific outcome of KY deficiency. Furthermore, KY shows protease activity in in vitro assays, and specific degradation of filamin C by KY is shown in transfected cells. Given the enzymatic nature of the KY protein, it is likely that some of the identified partners are catalytic substrates. These results suggest that KY is an intrinsic part of the protein networks underlying the molecular mechanism of several limb-girdle muscular dystrophies, particularly those where interactions between filamin C and disease causing proteins have been shown.

* To whom correspondence should be addressed at: MRC Mammalian Genetics Unit, Harwell, Didcot, OX11 0RD, UK. Tel: +44 1235841072; Fax: +44 1235841200; Email: gonzalo{at}har.mrc.ac.uk


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