Reactome: A Curated Pathway Database

alpha-aminoadipoate semialdehyde + NAD+ => alpha-aminoadipate + NADH + H+ (R-HSA-70941) [Homo sapiens]


Alpha-aminoadipic semialdehyde dehydrogenase (ALDH7A1) catalyzes the reaction of alpha-aminoadipic semialdehyde and NAD+ to form alpha-aminoadipate and NADH + H+ (Mills et al. 2006). Unpublished crystallographic data (PDB 2J6L) indicate that the enzyme is a homodimer. Recent immunofluorescence studies of both endogenous and GFP-tagged ALDH7A1 proteins in cultured human embryonic kidney cells indicate that the protein is present in both mitochondria and the cytosol (Wong et al. 2010).

Additional Information
Compartment mitochondrial matrix
Components of this entry
Input entries
Output entries
Catalyst Activity
PhysicalEntity Activity Active Units
ALDH7A1 tetramer L-aminoadipate-semialdehyde dehydrogenase activity (0004043)  
Cross References
Database Identifier
Rhea 12309, 12311
Literature References
pubMedId Title Journal Year
16491085 Mutations in antiquitin in individuals with pyridoxine-dependent seizures Nat Med 2006
19885858 Is antiquitin a mitochondrial Enzyme? J Cell Biochem 2010