Very Long Chain Fatty Acids

The Very Long Chain Fatty Acids test contains 1 test with 11 biomarkers.

Clinical Significance

Peroxisomes play a vital role in several metabolic pathways, including the synthesis of plasmalogens and bile acids. They are also involved in the catabolism of Very Long Chain Fatty Acids (VLCFAs), phytanic acid, and pristanic acid. Defects in these pathways usually result in the accumulation in tissues and body fluids of one or more metabolites derived from the blocked metabolic steps. Specific accumulations are used for the differential biochemical diagnosis of numerous peroxisomal disorders. These disorders include (A) Zellweger spectrum disorders of peroxisomal biogenesis, (B) X-linked adrenoleukodystrophy (X-ALD) and its adult form X-linked adrenomyeloneuropathy (X-AMN), (C) Refsum disease (Phytanoyl-CoA hydroxylase Deficiency), and (D) 2-methylacyl-CoA racemase deficiency.

Patient Preparation 

Avoid alcohol for 24 hours prior to collection. Draw sample in the morning following overnight fasting (12-14 hours)

The following is a list of what is included in the item above. Click the test(s) below to view what biomarkers are measured along with an explanation of what the biomarker is measuring.

Age

Discriminant Function

Docosanoic Acid, C22

Gender

Hexacosanoic Acid, C26

Phytanic Acid

Pristanic Acid

Ratio C24/C22

Ratio C26/C22

Ratio Pristanic/Phytanic

Tetracosanoic Acid, C24

*Process times are an estimate and are not guaranteed. The lab may need additional time due to weather, holidays, confirmation/repeat testing, or equipment maintenance.

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