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Biotinidase

Blood Sciences Test


Specimen

EDTA plasma

Special Instructions

Must arrive into the lab within 6 hrs of sample collection

Units

umol/L/min

Test Usage

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.

Turnaround time

14 days

Availability

Referred test

Cannot be added on to an existing request

Specimen Labelling Procedure
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