Limitations of 3-phenylpropionylglycine in early screening for medium-chain acyl-coenzyme A dehydrogenase deficiency.
Screening for medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency by urinary 3-phenylpropionylglycine may not be reliable in early infancy because young infants are not colonized with adult-type colonic flora. In this study we delineated the microbes that produce 3-phenylpropionic acid, the precursor of 3-phenylpropionylglycine. We found that the use of some antibiotics may alter gut flora, thereby confounding this method of screening for MCAD deficiency.
Bhala
A
A
Department of Pediatrics and Pathology, University of Pennsylvania School of Medicine, Philadelphia.
Bennett
M J
MJ
McGowan
K L
KL
Hale
D E
DE
D042964:Acyl-CoA Dehydrogenase; D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000494:pharmacology; D001421:Bacteria, Anaerobic / Q000187:drug effects / Q000378:metabolism*; D002208:Caproates / Q000378:metabolism; D002648:Child; D003013:Clostridium / Q000378:metabolism; D044943:Fatty Acid Desaturases / Q000172:deficiency*; D005998:Glycine / Q000031:analogs & derivatives* / Q000096:biosynthesis / Q000652:urine; D006801:Humans; D007223:Infant; D010649:Phenylalanine / Q000378:metabolism; D010666:Phenylpropionates / Q000378:metabolism
D000900:Anti-Bacterial Agents; D002208:Caproates; D010666:Phenylpropionates; C037652:hexanoic acid; C057466:3-phenylpropionylglycine; C024571:N-caproylglycine; D010649:Phenylalanine; C035253:3-phenylpropionic acid; D044943:Fatty Acid Desaturases; D042964:Acyl-CoA Dehydrogenase; D005998:Glycine