Neurotrophin-4 is up-regulated in ragged-red fibers associated with pathogenic mitochondrial DNA mutations

Annals of Neurology
U A Walker, E A Schon

Abstract

Ragged-red fibers (RRFs) are found more frequently in highly oxidative type I fibers than in glycolytic type II fibers in the muscle of many patients with mitochondrial myopathies. Neurotrophin-4 (NT-4), a neuronal signaling molecule, is also expressed in skeletal muscle, predominantly in type I fibers. We found that NT-4 protein and mRNA were present in both type I and type II fibers but were up-regulated in RRFs of patients with mitochondrial encephalomyopathies; it is noteworthy that NT-4 was not up-regulated in muscle fibers from healthy aerobically trained athletes. Thus, NT-4 might represent a member of a new class of candidate molecules involved in the compensatory adjustments of muscle fibers to oxidative dysfunction, and may even play a role as a signaling molecule for mitochondrial proliferation.

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Citations

Mar 24, 2004·Cancer Genetics and Cytogenetics·Stefanie ZanssenEric A Schon
Nov 21, 2000·Trends in Biochemical Sciences·E A Schon
Oct 1, 2011·Journal of Biomedicine & Biotechnology·Kunihiro Sakuma, Akihiko Yamaguchi
Oct 9, 2009·Muscle & Nerve·Daniel I Ogborn, Phillip F Gardiner
Feb 17, 2009·The Neuroscientist : a Review Journal Bringing Neurobiology, Neurology and Psychiatry·John GardinerJan Marc
Oct 18, 2005·Muscle & Nerve·Guillaume ChevrelMichael Sendtner
Dec 16, 1998·Current Opinion in Neurology·E A Shoubridge
Jan 17, 2020·Journal of Applied Physiology·Miguel Pareja-CajiaoCarlos B Mantilla

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