title: The effect of a high protein diet on leucine and alanine turnover in acid maltase deficiency.
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.
authors.affiliation: Department of Medicine, St Thomas' Hospital, London, UK.
authors.forename: A M
authors.initials: AM
authors.lastname: Umpleby
authors.forename: P S
authors.initials: PS
authors.lastname: Trend
authors.forename: D
authors.initials: D
authors.lastname: Chubb
authors.forename: J V
authors.initials: JV
authors.lastname: Conaglen
authors.forename: C D
authors.initials: CD
authors.lastname: Williams
authors.forename: R
authors.initials: R
authors.lastname: Hesp
authors.forename: I N
authors.initials: IN
authors.lastname: Scobie
authors.forename: C M
authors.initials: CM
authors.lastname: Wiles
authors.forename: G
authors.initials: G
authors.lastname: Spencer
authors.forename: P H
authors.initials: PH
authors.lastname: Sönksen
mesh_terms: D000328:Adult; D000409:Alanine / Q000097:blood*; D002986:Clinical Trials as Topic; D004044:Dietary Proteins / Q000008:administration & dosage*; D005260:Female; D005500:Follow-Up Studies; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency*; D005951:Glucose Tolerance Test; D006009:Glycogen Storage Disease Type II / Q000178:diet therapy* / Q000201:enzymology; D006801:Humans; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D007930:Leucine / Q000097:blood*; D008297:Male; D008875:Middle Aged; D011897:Random Allocation; D000520:alpha-Glucosidases
chemical_list: D004044:Dietary Proteins; D007773:Lactates; D019344:Lactic Acid; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase; D007930:Leucine; D000409:Alanine