title: Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia.
abstract: The rates of propionate production and aminoacid catabolism in 5 children with methylmalonic acidaemia were measured by use of stable isotope techniques. Total propionate production was 55-186 mumol/kg per h, to which the maximum contribution of protein catabolism was 10-35 mumol/kg per h (5-40%). These findings indicate important sources of propionate other than protein catabolism, which may account for the limited efficacy of dietary protein restriction in treatment of methylmalonic acidaemia.
authors.affiliation: Department of Child Health, Institute of Child Health, London.
authors.forename: J H
authors.initials: JH
authors.lastname: Walter
authors.forename: G N
authors.initials: GN
authors.lastname: Thompson
authors.forename: J V
authors.initials: JV
authors.lastname: Leonard
authors.forename: K
authors.initials: K
authors.lastname: Bartlett
authors.forename: D
authors.initials: D
authors.lastname: Halliday
mesh_terms: D000214:Acyl Coenzyme A / Q000096:biosynthesis*; D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood*; D000596:Amino Acids / Q000378:metabolism*; D002648:Child; D006801:Humans; D007532:Isoleucine / Q000378:metabolism; D007700:Kinetics; D007930:Leucine / Q000378:metabolism; D008314:Malonates / Q000097:blood*; D008715:Methionine / Q000378:metabolism; D008764:Methylmalonic Acid / Q000097:blood*; D010649:Phenylalanine / Q000378:metabolism; D013912:Threonine / Q000378:metabolism; D013997:Time Factors; D014633:Valine / Q000378:metabolism
chemical_list: D000214:Acyl Coenzyme A; D000596:Amino Acids; D008314:Malonates; D007532:Isoleucine; D013912:Threonine; C009061:propionyl-coenzyme A; D010649:Phenylalanine; D008764:Methylmalonic Acid; D008715:Methionine; D007930:Leucine; D014633:Valine