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Lipoamidase activity in human serum is due to biotinidase.

Abstract
Lipoamidase, as determined by lipoyl-p-aminobenzoic acid (L-pABA) hydrolyzing activity, and biotinidase in human serum have similar pH profiles, molecular weights, thermostabilities, and are similarly inhibited by p-hydroxymercuribenzoate and not inhibited by phenylmethylsulfonylfluoride. A monospecific polyclonal antibody prepared against biotinidase immunoprecipitated greater than 95% of serum L-pABA hydrolyzing activity and an identical proportion of biotinidase activity. In addition, children with profound biotinidase deficiency (less than 10% normal serum activity) have greatly reduced levels of L-pABA hydrolyzing activity in serum (less than 15% of mean normal activity) and obligate heterozygotes have activities intermediate between that of normal and profoundly deficient individuals. These results indicate that most, if not all, of the L-pABA hydrolyzing activity in human serum is due to biotinidase. Moreover, since the Km of L-pABA hydrolysis by serum is high, it is unlikely that lipoic acid is recycled in the serum by biotinidase.
AuthorsC L Garganta, B Wolf
JournalClinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 189 Issue 3 Pg. 313-25 (Aug 31 1990) ISSN: 0009-8981 [Print] Netherlands
PMID2225462 (Publication Type: Comparative Study, Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Aminobenzoates
  • Hydroxymercuribenzoates
  • para-Aminobenzoates
  • lipoyl-4-aminobenzoic acid
  • 4-hydroxymercuribenzoate
  • N-biotinyl-4-aminobenzoic acid
  • Phenylmethylsulfonyl Fluoride
  • Biotin
  • Thioctic Acid
  • Amidohydrolases
  • lipoamidase
  • Biotinidase
  • 4-Aminobenzoic Acid
Topics
  • 4-Aminobenzoic Acid (blood)
  • Amidohydrolases (blood, metabolism)
  • Aminobenzoates
  • Biotin (analogs & derivatives, blood)
  • Biotinidase
  • Chromatography, Gel (methods)
  • Enzyme Stability
  • Hot Temperature
  • Humans
  • Hydrogen-Ion Concentration
  • Hydrolysis
  • Hydroxymercuribenzoates (pharmacology)
  • Kinetics
  • Milk, Human (enzymology, metabolism)
  • Molecular Weight
  • Phenylmethylsulfonyl Fluoride (pharmacology)
  • Thioctic Acid (analogs & derivatives, blood)
  • para-Aminobenzoates

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