Article
Cell stress molecules in the skeletal muscle of GNE myopathy.
BMC neurology - 12 Mar 2013
Fischer Charlotte, Kleinschnitz Konstanze, Wrede Arne, Muth Ingrid, Kruse Niels, Nishino Ichizo, Schmidt Jens
Abstract excerpt
BACKGROUND: Mutations of the UDP-N-acetylglucosamine-2-epimerase/N-acetylmannosamine-kinase (GNE)-gene are causally related to GNE myopathy. Yet, underlying pathomechanisms of muscle fibre damage have remained elusive. In sporadic inclusion body myositis (sIBM), the pro-inflammatory cell-stress mediators αB-crystallin and inducible nitric oxide synthase (iNOS) are crucial markers of the disease pathology....
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