Biotinidase (BTD)
Serum biotinidase (BTD; EC 3.5.1.12) catalyzes the hydrolysis of biocytin, a normal product of biotin-dependent carboxylase degradation, to biotin and lysine. The process results in the regeneration of free biotin. Biotin is an essential water-soluble vitamin and is the coenzyme for 4 carboxylases necessary for normal metabolism in humans: pyruvate carboxylase (PCC), propionyl-CoA carboxylase (PCCA), alpha-methylcrotonyl-CoA carboxylase , and acetyl-CoA carboxylase (ACACA).
The deduced 543-amino acid protein has a molecular mass of approximately 57 kD and contains 6 potential N-linked glycosylation sites. Northern blot analysis detected BTD mRNA in human heart, brain, placenta, liver, lung, skeletal muscle, kidney, and pancreas.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPJ527Hu01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAJ527Hu01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
MAJ527Hu21 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
Assay Kits SEJ527Hu ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Biotinidase (BTD) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Biotinidase (BTD) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Biotinidase (BTD) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Biotinidase (BTD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Biotinidase (BTD) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Biotinidase (BTD) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Biotinidase (BTD) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Biotinidase (BTD) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Biotinidase (BTD) CLIA Kit Customized Service Offer
n/a ELISA Kit for Biotinidase (BTD) ELISA Kit Customized Service Offer
  1. "Human serum biotinidase. cDNA cloning, sequence, and characterization."J. Biol. Chem. 269:6566-6570(1994) [PubMed] [Europe PMC] [Abstract]
  2. "Structure of the human biotinidase gene."Mamm. Genome 9:327-330(1998) [PubMed] [Europe PMC] [Abstract]
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  4. "The DNA sequence, annotation and analysis of human chromosome 3." Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
  5. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  6. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
  7. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
  8. "A strategy for precise and large scale identification of core fucosylated glycoproteins."Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]
  9. "Arg538 to Cys mutation in a CpG dinucleotide of the human biotinidase gene is the second most common cause of profound biotinidase deficiency in symptomatic children."Hum. Genet. 99:506-512(1997) [PubMed] [Europe PMC] [Abstract]
  10. "Partial biotinidase deficiency is usually due to the D444H mutation in the biotinidase gene."Hum. Genet. 102:571-575(1998) [PubMed] [Europe PMC] [Abstract]
  11. "Double mutation (A171T and D444H) is a common cause of profound biotinidase deficiency in children ascertained by newborn screening in the United States."Hum. Mutat. 11:410-410(1998) [PubMed] [Europe PMC] [Abstract]