Table 3.

List of known ALDH enzymes annotated with substrate preference, genetics, and cellular localization, where known

EnzymeLikely substrateTissue expressionAdult deficiencyLocalization
ALDH1a1 9-Cis retinaldehyde, maybe all-trans. Cyclophosphamide metabolite All tissues: adipose, thyroid, liver, astrocyte enriched Healthy and resistant to obesity Cytosol 
ALDH1a2 All-trans retinaldehyde only Reproductive tissues and immune tissues Healthy Cytosol 
ALDH1a3 All-trans retinaldehyde Prostate/salivary glands Healthy Cytosol 
ALDH2 Acetaldehyde Liver Alcohol aversion, neurological and motor function Mitochondria 
ALDH1L1 10-Formyl-THF Liver Unknown, likely lethal Cytosol 
ALDH1L2 10-Formyl-THF Pancreas Unknown, likely lethal Mitochondria 
ALDH1b1 Acetaldehyde All tissues Increased steatosis Mitochondria 
ALDH3a1 Long chain lipid aldehydes, UV-based decay products, oxazaphosphorines, smoking damage Cornea, esophagus, stomach Viable; predisposed to cataracts Cytosol 
ALDH3a2 Long chain lipid aldehydes All tissues Sjogren-Larsson syndrome Cytosol 
ALDH3b1 Long chain lipid aldehydes All tissues Unknown Cytosol 
Aldh3b2 Long chain lipid aldehydes Breast, esophagus, skin Unknown Cytosol 
ALDH4a1 Glutamate semialdehyde Kidney, liver 4-hydroxybutyric aciduria Mitochondria 
ALDH5a1 Succinate semialdehyde All tissues 4-hydroxybutyric aciduria Mitochondria 
ALDH6a1 Methylmalonate semialdehyde All tissues Demyelination and methylmalonic aciduria Mitochondria 
ALDH7a1 Aminoadipic semialdehyde All tissues Pyridoxine-dependent epilepsy Mixed 
ALDH8a1 2-Aminomuconate semialdehyde, potential 9-cis retinal Kidney, liver Unknown Cytosol 
ALDH9a1 GABA All tissues Unknown Cytosol 
ALDH16a1 Non-catalytic Low basal expression Unknown Cytosol 
ALDH18a1 Pyrroline-5-carboxylate All tissues Cutis laxia Mitochondria 
EnzymeLikely substrateTissue expressionAdult deficiencyLocalization
ALDH1a1 9-Cis retinaldehyde, maybe all-trans. Cyclophosphamide metabolite All tissues: adipose, thyroid, liver, astrocyte enriched Healthy and resistant to obesity Cytosol 
ALDH1a2 All-trans retinaldehyde only Reproductive tissues and immune tissues Healthy Cytosol 
ALDH1a3 All-trans retinaldehyde Prostate/salivary glands Healthy Cytosol 
ALDH2 Acetaldehyde Liver Alcohol aversion, neurological and motor function Mitochondria 
ALDH1L1 10-Formyl-THF Liver Unknown, likely lethal Cytosol 
ALDH1L2 10-Formyl-THF Pancreas Unknown, likely lethal Mitochondria 
ALDH1b1 Acetaldehyde All tissues Increased steatosis Mitochondria 
ALDH3a1 Long chain lipid aldehydes, UV-based decay products, oxazaphosphorines, smoking damage Cornea, esophagus, stomach Viable; predisposed to cataracts Cytosol 
ALDH3a2 Long chain lipid aldehydes All tissues Sjogren-Larsson syndrome Cytosol 
ALDH3b1 Long chain lipid aldehydes All tissues Unknown Cytosol 
Aldh3b2 Long chain lipid aldehydes Breast, esophagus, skin Unknown Cytosol 
ALDH4a1 Glutamate semialdehyde Kidney, liver 4-hydroxybutyric aciduria Mitochondria 
ALDH5a1 Succinate semialdehyde All tissues 4-hydroxybutyric aciduria Mitochondria 
ALDH6a1 Methylmalonate semialdehyde All tissues Demyelination and methylmalonic aciduria Mitochondria 
ALDH7a1 Aminoadipic semialdehyde All tissues Pyridoxine-dependent epilepsy Mixed 
ALDH8a1 2-Aminomuconate semialdehyde, potential 9-cis retinal Kidney, liver Unknown Cytosol 
ALDH9a1 GABA All tissues Unknown Cytosol 
ALDH16a1 Non-catalytic Low basal expression Unknown Cytosol 
ALDH18a1 Pyrroline-5-carboxylate All tissues Cutis laxia Mitochondria 

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