Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia.
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.
Walter
J H
JH
Department of Child Health, Institute of Child Health, London.
Thompson
G N
GN
Leonard
J V
JV
Bartlett
K
K
Halliday
D
D
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
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