PMID: 2507747Aug 1, 1989Paper

The effect of a high protein diet on leucine and alanine turnover in acid maltase deficiency

Journal of Neurology, Neurosurgery, and Psychiatry
A M UmplebyP H Sonksen

Abstract

Leucine and alanine production rate was measured in 5 patients with acid maltase deficiency in the postabsorptive state, following 6 months on a normal diet with placebo and 6 months on an isocaloric high protein diet (16-22% protein). Whole body leucine production rate, a measure of protein degradation, expressed in terms of lean body mass was significantly greater than in five control subjects. Following the high protein diet, leucine production rate was decreased in four of the five patients but this was not statistically significant. Alanine production rate expressed in terms of lean body mass was significantly greater than in control subjects. After the high protein diet, alanine production rate and concentration were significantly decreased (p less than 0.05). There were no significant improvements in any of the clinically relevant variables measured in these patients. It is possible that a larger increase in protein intake over a longer time period may have a clinical effect.

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Citations

Apr 8, 2006·Journal of Inherited Metabolic Disease·H R MundyP J Lee
May 17, 2006·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Laura Elizabeth Case, Priya Sunil Kishnani
Jul 24, 2008·Journal of Neuropathology and Experimental Neurology·Richard L SidmanLamya S Shihabuddin
Apr 9, 2016·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Mark TarnopolskyAneal Khan
Sep 18, 2012·Molecular Genetics and Metabolism·Nicolai PreislerJohn Vissing
Dec 2, 2005·Muscle & Nerve·Alfred E SlonimSalvatore DiMauro

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