Aldehyde dehydrogenase 9 family member A1 (ALDH9A1)

Aldehyde dehydrogenase 9 family member A1 (ALDH9A1) is a cytosolic, tetrameric NAD^+-dependent enzyme belonging to the aldehyde dehydrogenase superfamily[1][2]. It exhibits broad substrate specificity for various aminoaldehydes and is primarily responsible for oxidizing 4-N-trimethylaminobutyraldehyde (TMABAL) in the carnitine biosynthesis pathway—a process essential for fatty acid transport into mitochondria via β-oxidation and cellular energy metabolism[1][2]. ALDH9A1 also participates in detoxifying other aldehydes, potentially contributing to gamma-aminobutyric acid (GABA) production and maintaining metabolic equilibrium. Its gene expression is regulated by the peroxisome proliferator-activated receptor alpha (PPARα), linking it to broader metabolic and energetic regulation in tissues with high β-oxidation rates, such as liver and kidney[2]. No drugs specifically targeting ALDH9A1 or its clinical biomarker roles are currently described in the provided references.

Other names

4-trimethylaminobutyraldehyde dehydrogenaseTMABA-DHTMABALDHE3Aldehyde dehydrogenase E3 isozymeFormaldehyde dehydrogenaseGamma-aminobutyraldehyde dehydrogenaseR-aminobutyraldehyde dehydrogenaseALDH9TMABADH