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Old 03-20-2011
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Biochemistry Why do they call them LCAD and MCAD

Any body know why fatty acyl-Coa dehydrogenase is named as LCAD or MCAD?

thanks!
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Old 03-20-2011
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OK, I did some search and here is the answer:

LCAD: long chain acyl-CoA dehydrogenase
MCAD: medium chain acyl-CoA dehydrogenase

Deficiency of either one cause faster hypoglycemia
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Old 03-20-2011
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Correct Answer

Quote:
Originally Posted by usluipek View Post
Any body know why fatty acyl-Coa dehydrogenase is named as LCAD or MCAD?

thanks!
Fatty Acyl CoA Dehydrogenase has many forms -

LCAD - long chain fatty acyl coA dehydrogenase
MCAD - medium chain fatty acyl coA dehydrogenase
SCAD - small chain fatty acyl coA dehydrogenase

They are used in the Beta Oxidation reaction in the Mitochondrial Matrix. There names are so because each of them are used for a different lengths of Fatty Acids. LCAD for fatty acids with more than 10 Carbon chains, MCAD for 8 to 10 carbon chains and SCAD to finish the job with less than 7 carbon chains.

The deficiency causes Fasting Hypoglycemia and hypoketosis (since no ketone bodies are produced). Other symptoms include: lethargy, vomiting, coma, and death. It is Autosomal Recessive with variable expression.

Also, note that since this is occurring in a fasting stage the body then utilizes Omega Oxidation (methyl carbon oxidation) from Peroxisomes to generate energy which produces Dicarboxylic Acid. Therefore, you will also see dicarboxyacedmia in the blood work.

The primary treatment is IV Glucose.

Prevention is frequent feeding, high-carbohydrate and low-fat diet.


Hope this helps.
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