biocytinase

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biocytinase

[‚bī·ō′sīt·ən‚ās]
(biochemistry)
An enzyme present in the blood and liver which hydrolyzes biocytin into biotin and lysine.
References in periodicals archive ?
5431, which specifies that a health professional in charge of the care of or at the birth of an infant shall administer or cause to be administered a test for "(a) Phenylketonuria; (b) Galactosemia; (c) Hypothyroidism; (d) Maple syrup urine disease; (e) Biotinidase deficiency; (f) Sickle cell anemia; (g) Congenital adrenal hyperplasia; (h) Other treatable but otherwise disabling conditions as designated by the department.
Finally, the biotin-tag was protected from serum biotinidase activity by incorporation of 2-amino-butyric acid (27, 30) to avoid the use of iodacetamid, which inhibits not only biotinidase but also cysteine proteases (31).
The metabolic disorders on the March of Dimes-recommended list for screening are: phenylketonuria (PKU); congenital hypothyroidism; congenital adrenal hyperplasia (CAH); biotinidase deficiency; maple syrup urine disease; galactosemia; homocystinuria; sickle cell anemia; and medium-chain acyl-CoA dehydrogenase (MCAD) deficiency.
The metabolic disorders on the recommended list for screening are: phenylketonuria (PKU); congenital hypothyroidism; congenital adrenal hyperplasia (CAH); biotinidase deficiency; maple syrup urine disease; galactosemia; homocystinuria; sickle cell anemia; and medium-chain acyl-CoA dehydrogenase (MCAD) deficiency.
The development of MS/MS has helped to reduce the price of newborn screening, since it can test for nearly 30 different disorders all at once, including conditions such as PKU, maple syrup urine disease, and biotinidase.
Use of filter-paper blood spots from newborns for screening of inborn errors may include the assay of biotinidase (EC 3.
In July, the state added newborn screening for biotinidase deficiency, congenital adrenal hyperplasia (CAH), cystic fibrosis, and maple syrup urine disease.
It is noteworthy that a small fraction of infants with biotinidase deficiency will have increased C50H.
Along with MCAD testing, the March of Dimes' recommended newborn screening test list includes: phenylketonuria (PKU), congenital hypothyroidism, galactosemia, sickle cell anemia, congenital adrenal hyperplasia (CAH), biotinidase deficiency, maple syrup urine disease, homocystinuria and a hearing test.
Of these 8 tests, it is currently up to state public health agencies whether they also screen for maple syrup urine disease, homocystinuria, biotinidase deficiency, and congenital adrenal hyperplasia.
DBS are also used routinely for enzyme measurements within newborn-screening programs for the detection of biotinidase and galactose-uridyltransferase deficiencies in neonates.