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Biotinidase (BTD)

Biotinase

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)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Hu01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Hu01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Hu01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Hu01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Hu01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Mu01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Mu01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Mu01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Mu01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Mu01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Ra01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Ra01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Ra01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Ra01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Ra01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Ca01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Ca01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Ca01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Ca01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Ca01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Po01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Po01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Po01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Po01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Po01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Bo01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Bo01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Bo01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Bo01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Bo01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Rb01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Rb01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Rb01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Rb01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Rb01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPO222Mo01 Recombinant Biotinidase (BTD) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAO222Mo01 Monoclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IP.
PAO222Mo01 Polyclonal Antibody to Biotinidase (BTD) WB; IHC; ICC; IF.
Assay Kits CLO222Mo01 CLIA Kit for Biotinidase (BTD) CLIA assay for Antigen Detection
ELO222Mo01 ELISA Kit for Biotinidase (BTD) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

"Human serum biotinidase. cDNA cloning, sequence, and characterization."
J. Biol. Chem. 269:6566-6570(1994) [PubMed] [Europe PMC] [Abstract]

"Structure of the human biotinidase gene."
Mamm. Genome 9:327-330(1998) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence, annotation and analysis of human chromosome 3."
Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]

"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]

"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]

"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]

"A strategy for precise and large scale identification of core fucosylated glycoproteins."
Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]

"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]

"Partial biotinidase deficiency is usually due to the D444H mutation in the biotinidase gene."
Hum. Genet. 102:571-575(1998) [PubMed] [Europe PMC] [Abstract]

"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]