Disease relevance of Btd

• In this study we aimed to put forward the biotinidase deficiency considered as a possible cause of alopecia in the rats administered with valproic acid, and the correlation between liver and serum biotinidase enzyme activities (BEA) and transaminases, albumin and serum valproic acid levels [1].
• Decrease in plasma biotinidase activity with normal albumin concentrations in experimental liver fibrosis [2].
• Rat as a potential model for hearing loss in biotinidase deficiency [3].

High impact information on Btd

• Extension of the single amino acid chelate concept (SAAC) to bifunctional biotin analogues for complexation of the M(CO)3(+1) Core (M = Tc and Re): syntheses, characterization, biotinidase stability, and avidin binding [4].
• TcL6 and TcL9 were resistant to biotinidase cleavage, while TcL12a, which contains a lysine linkage, was rapidly cleaved [4].
• Neuronal biocytin uptake might contribute to the pathogenesis of biotinidase deficiency where biocytin is present in elevated levels [5].
• Biotinidase in the porcine cerebrum [6].
• Subcellular distribution of biotinidase was found mainly in the soluble cytoplasmic fraction (S3), i.e., in the supernatant of 0.32 M sucrose S2 solution after ultracentrifugation at 105,000g for 90 min [6].

Biological context of Btd

• Lipoamidase and biotinidase activities in the rat: tissue distribution and intracellular localization [7].

Anatomical context of Btd

• Fractionation of solubilized brush border preparations indicates that biotinidase is the only protein which binds biotin in this preparation [8].
• Lipoyllysine hydrolase and biotinidase activities were highest in kidney, liver and blood plasma, whereas lipoyl-p-aminobenzoic acid hydrolase activities were highest in liver, brain and kidney [7].

Associations of Btd with chemical compounds

• Serum and liver tissue biotinidase enzyme activity in rats which were administrated to valproic acid [1].
• In the plasma of ethanol/phenobarbital-treated rats as well as the ethanol-treated rats total thiol, albumin and the activity of biotinidase were decreased significantly as compared with the controls [9].
• A marked decrease in the activity of biotinidase in the plasma of rats after single dose of carbon tetrachloride [10].
• The activities of glutamine synthase and biotinidase were decreased significantly in the livers of ethanol/phenobarbital-treated rats as well as the ethanol-treated rats as compared with the controls [9].
• Lipoyllysine hydrolase and biotinidase activities were found mainly in the liver microsomal fraction, and lipoyl-p-aminobenzoic acid hydrolase was recovered from the microsomal fraction and to a small extent from the mitochondrial fraction [7].

Analytical, diagnostic and therapeutic context of Btd

• However, biotinylated A beta1-40 peptide radiopharmaceuticals conjugated to the RI7/SA brain drug targeting system were metabolically unstable in mice in vivo owing to active biotinidase activity [11].

References