BTD Rabbit pAb, FITC conj. (标记一抗) | Bioss

bs-11813R-FITC

BTD Rabbit pAb, FITC conj.

FITC标记的生物素酶抗体

Biotinase; Biotinidase; Btd; Sp8; BTD_HUMAN; EC 3.5.1.12.

FITC
Excitation spectrum: 495nm
Emission spectrum: 519nm

Rabbit

KLH conjugated synthetic peptide derived from human Biotinidase: 401-500/543

IgG

affinity purified by Protein A

Polyclonal

57 kDa

1mg/ml

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

Biotin, also known as vitamin B7, is an essential water-soluble vitamin that is a cofactor in glucogenesis and in the metabolism of fatty acids and leucine. Biotinidase is a 523 amino acid enzyme that catalyzes the hydrolysis of biocytin to biotin and lysine. Secreted into extracellular space, biotinidase is expressed in liver, heart, placenta, brain, skeletal muscle, pancreas and kidney. Biotinidase contains one carbon-nitrogen hydrolase domain, which is involved in the reduction of organic nitrogen compounds and ammonia production. Defects in the gene encoding biotinidase are the cause of biotinidase deficiency, which is characterized by skin rash, ataxia, seizures, hearing loss, hypotonia and optic atrophy. These symptoms are due to the individual’s inability to reutilize biotin and can, therefore, typically be treated with the addition of free biotin.

BTD

Biotinidase

Secreted.

Defects in BTD are the cause of biotinidase deficiency (BTD deficiency) [MIM:253260]; also called late-onset multiple carboxylase deficiency. BTD deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis.
BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur.

Belongs to the CN hydrolase family. BTD/VNN subfamily.
Contains 1 CN hydrolase domain.

Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.