A bioassay for determining biotinidase activity and for discriminating biocytin from biotin using holocarboxylase synthetase-deficient cultured fibroblasts

J Inherit Metab Dis. 1985:8 Suppl 2:101-2. doi: 10.1007/BF01811478.
No abstract available

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amidohydrolases / analysis*
  • Amidohydrolases / deficiency
  • Biological Assay
  • Biotin / metabolism*
  • Biotinidase
  • Carbon-Nitrogen Ligases*
  • Carboxy-Lyases / analysis
  • Cells, Cultured
  • Fibroblasts / enzymology
  • Humans
  • Ligases / deficiency*
  • Lysine / analogs & derivatives*
  • Lysine / metabolism
  • Methylmalonyl-CoA Decarboxylase

Substances

  • Biotin
  • Amidohydrolases
  • Biotinidase
  • Carboxy-Lyases
  • Ligases
  • Carbon-Nitrogen Ligases
  • holocarboxylase synthetases
  • Methylmalonyl-CoA Decarboxylase
  • biocytin
  • Lysine