Must arrive into the lab within 6 hrs of sample collection
It is used in the investigation of biotin responsive disorders, i.e. glycinaemia, propionic acidaemia, ß-OH isovaleric aciduria and ß-methyl crotonglycinuria.
Propionyl Co-A, formed in the catabolism of several essential amino acids is itself converted to succinyl Co-A by a series of reactions, one of which, catalysed by propionyl Co-A carboxylase requires biotin as co-factor.
There are in fact four enzyme systems that require biotin, three being catabolic producing succinyl Co-A from amino acids, the fourth, involving acetyl Co-A carboxylase, leads to the production of long-chain fatty acids.
Biotin deficiency, however, is almost never reported in humans.
Cannot be added on to an existing requestSpecimen Labelling Procedure