Myelin proteins: degradation in rat brain initiated by metabolites causative of maple syrup urine disease

Biochemical and Biophysical Research Communications
D Tribble, R Shapira

Abstract

Maple syrup urine disease (MSUD), an inborn error of metabolism in humans, is expressed as an inability to oxidatively decarboxylate the branched-chain alpha-keto acids derived from leucine, isoleucine and valine. Rats 14 days old were injected intracranially with a solution containing leucine, alpha-ketoisocaproate, and tracer amounts of 3H-lysine. Myelin isolated from these rat brains at 28 days of age had a washed dry weight 85 per cent of controls. The protein content of the myelin prepared from treated and control rats was identical, as were the specific activities of the individual proteins separated by polyacrylamide gel electrophoresis. Myelin protein from treated rats was deficient in myelin high molecular weight proteins including glycoproteins, and degradation products of these proteins were observed in myelin of treated rats. MSUD associated metabolites in man may initiate a process leading to the proteolytic degradation of myelin proteins, thereby producing abnormal myelin sheaths.

Citations

Jan 16, 2004·Pharmacology, Biochemistry, and Behavior·Vilson de Castro VasquesMoacir Wajner
Nov 14, 2013·Cellular and Molecular Neurobiology·Angela SittaCarmen R Vargas
Jun 15, 2014·Metabolism: Clinical and Experimental·Janne M StrandLars Eide
Aug 21, 2003·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Raquel BridiCarlos Severo Dutra-Filho
Feb 14, 2012·Molecular Neurobiology·Giselli ScainiEmilio L Streck
May 15, 2007·Journal of the Neurological Sciences·Cláudia FunchalMoacir Wajner
Nov 26, 2003·Biochimica Et Biophysica Acta·Angela M SgaravattiMoacir Wajner
Nov 9, 2006·Metabolic Brain Disease·Alethéa G BarschakCarmen R Vargas
Nov 21, 2007·Metabolic Brain Disease·Alethéa G BarschakCarmen R Vargas

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