Isolated biotin-resistant 3-methylcroton
โ
F. A. Beemer; K. Bartlett; M. Duran; H. K. Ghneim; S. K. Wadman; L. Bruinvis; D.
๐
Article
๐
1982
๐
Springer
๐
English
โ 476 KB
Two Vietnamese siblings with an isolated deficiency of 3-methylcrotonyl coenzyme A carboxylase in leucocytes and cultured fibroblasts are described. Both children excreted massive amounts of 3-methylcrotonylglycine and 3-hydroxyisovaleric acid. There was no in vivo or in vitro biochemical response t