Gene Information
Gene NameAASS
OrganismHomo sapiens (Human)
Gene Length926
Protein NamesAlpha-aminoadipic semialdehyde synthase, mitochondrial (LKR/SDH) [Includes: Lysine ketoglutarate reductase (LKR) (LOR) (EC 1.5.1.8); Saccharopine dehydrogenase (SDH) (EC 1.5.1.9)]
Target Namealpha-aminoadipic semialdehyde synthase, mitochondrial
Target TypeNA
Gene Age544 ( ENSG00000008311 )
Evolutionary StageEukaryota
AlphaFoldDBQ9UDR5
Gene CardAASS
Uniprot IDQ9UDR5
PfamPF01262; PF05222; PF16653; PF03435
In ASDno
Allosteric Predictionyes (0.210732791)
Ortholog in AnimalsAlpaca_ AASS ( ortholog_one2one )
Dog_ AASS ( ortholog_one2one )
GuineaPig_ AASS ( ortholog_one2one )
Macaque_ AASS ( ortholog_one2one )
Mouse_ Aass ( ortholog_one2one )
Pig_ AASS ( ortholog_one2one )
Rat_ Aass ( ortholog_one2one )
Rabbit_ AASS ( ortholog_one2one )
OhnologsNA
ParalogsNA
TissueNA
Tissue SpecificityExpressed in all 16 tissues examined with highest expression in the liver.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 5L76  (X-ray  2.57A  5L78  (X-ray  2.68A  5O1N  (X-ray  2.28A  5O1O  (X-ray  2.48A  5O1P  (X-ray  1.90A 
DBSNP NA 
Subcellular location [CC]Mitochondrion {ECO:0000269|PubMed:463877}.
Mouse PG classificationNA
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