ALDH7A1

(redirected from Betaine aldehyde dehydrogenase)

ALDH7A1

A gene on chromosome 5q31 that encodes an aldehyde dehydrogenase involved in lysine catabolism in the mitochondrial matrix.

Molecular pathology
ALDH7A1 mutation are associated with pyridoxine-dependent epilepsy.
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Betaine is synthesized by a two-step oxidation of choline in which mitochondrial choline oxidase first catalyzes the production of betaine aldehyde, which is further oxidized by mitochondrial betaine aldehyde dehydrogenase to betaine (Dragolovich, 1994).