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J. Biochem, 2004, Vol. 135, No. 6 709-712
© 2004 The Japanese Biochemical Society

MOLECULAR BIOLOGY

Subcellular Localization of Fukutin and Fukutin-Related Protein in Muscle Cells

Hiroshi Matsumoto1,2, Satoru Noguchi1,3,*, Kazuma Sugie1, Megumu Ogawa1,3, Kumiko Murayama1, Yukiko K. Hayashi1 and Ichizo Nishino1,3

1 Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo 187-8502; 2 Department of Pediatrics, National Defense Medical College, Saitama, 359-8513; 3 Core Research for Evolutional Science and Technology (CREST), Japan Science and Technology Corporation, Saitama 332-0012

Fukuyama-type congenital muscular dystrophy and congenital muscular dystrophy 1C are congenital muscular dystrophies that commonly display reduced levels of glycosylation of {alpha}-dystroglycan in skeletal muscle. The genes responsible for these disorders are fukutin and fukutin-related protein (FKRP), respectively. Both gene products are thought to be glycosyltransferases, but their functions have not been established. In this study, we determined their subcellular localizations in cultured skeletal myocytes. FKRP localizes in rough endoplasmic reticulum, while fukutin localizes in the cis-Golgi compartment. FKRP was also localized in rough endoplasmic reticulum in skeletal muscle biopsy sample. Our data suggest that fukutin and FKRP may be involved at different steps in O-mannosylglycan synthesis of {alpha}-dystroglycan, and FKRP is most likely involved in the initial step in this synthesis.

* To whom correspondence should be addressed. Tel: +81-423412712; Fax: +81-423461742 Email: noguchi{at}ncnp.go.jp


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