Ketolytic Enzyme Testing

OHSU Service Code:

067-6373

CPT Codes:

82657

Lab Section:

Biochemical Genetics

Synonyms:

2-Methyl-3-Hydroxybutyric Aciduria; 3 Oxoacid CoA Transferase; Acetyl-CoA Acetyltransferase, Mitochondrial; Alpha-Methylacetoaceticaciduria; Beta-Kethothiolase Deficiency; Ketolytic Enzymes; Ketone Utilization; Ketothiolase Deficiency; Methylacetoacetyl-CoA Thiolase Deficiency; SCOT; SCOT deficiency; Succinyl-CoA:3-Ketoacid-Coenzyme A Transferase

Specimen Requirements:

2 confluent T25 flasks of fibroblasts.
For "requisition slips".

Units:

nmol/min-mg protein

Reference Range:

8.9 - 20.6 nmoles/min/mg protein

Critical Values:

<5% control activity

Comments: