Hydroxyacyl-Coenzyme A dehydrogenase

Hydroxyacyl-Coenzyme A dehydrogenase (HAD) is an enzyme that plays a crucial role in the metabolism of fatty acids. It is involved in the beta oxidation process, where it catalyzes the third step, converting L-3-hydroxyacyl CoA to 3-ketoacyl CoA.

Function

Hydroxyacyl-Coenzyme A dehydrogenase is a mitochondrial enzyme that catalyzes the oxidation of a wide variety of fatty acids, alcohols, and steroids. The enzyme is a tetramer of identical subunits and belongs to the family of oxidoreductases. It specifically acts on the CH-OH group of donor with NAD+ or NADP+ as acceptor.

Clinical significance

Mutations in the HAD gene can lead to deficiencies in the enzyme, which can result in metabolic diseases such as Leigh syndrome, a severe neurological disorder that typically arises in the first year of life. This condition is characterized by progressive loss of mental and movement abilities, leading to early death.

See also

• Coenzyme A
• Beta oxidation
• Leigh syndrome

References

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