Entity Details

Primary name AATM_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP00505
EntryNameAATM_HUMAN
FullNameAspartate aminotransferase, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length430
SequenceStatuscomplete
DateCreated1986-07-21
DateModified2021-06-02

Ontological Relatives

GenesGOT2

GO terms

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GOName
GO:0003723 RNA binding
GO:0004069 L-aspartate:2-oxoglutarate aminotransferase activity
GO:0005543 phospholipid binding
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
GO:0005886 plasma membrane
GO:0006094 gluconeogenesis
GO:0006103 2-oxoglutarate metabolic process
GO:0006107 oxaloacetate metabolic process
GO:0006531 aspartate metabolic process
GO:0006532 aspartate biosynthetic process
GO:0006533 aspartate catabolic process
GO:0006536 glutamate metabolic process
GO:0007565 female pregnancy
GO:0007595 lactation
GO:0009064 glutamine family amino acid metabolic process
GO:0009066 aspartate family amino acid metabolic process
GO:0009986 cell surface
GO:0014850 response to muscle activity
GO:0015908 fatty acid transport
GO:0016212 kynurenine-oxoglutarate transaminase activity
GO:0016597 amino acid binding
GO:0019470 4-hydroxyproline catabolic process
GO:0019550 glutamate catabolic process to aspartate
GO:0019551 glutamate catabolic process to 2-oxoglutarate
GO:0019899 enzyme binding
GO:0030170 pyridoxal phosphate binding
GO:0030315 T-tubule
GO:0031406 carboxylic acid binding
GO:0032868 response to insulin
GO:0032991 protein-containing complex
GO:0042802 identical protein binding
GO:0043204 perikaryon
GO:0043278 response to morphine
GO:0045471 response to ethanol
GO:0046487 glyoxylate metabolic process
GO:0070062 extracellular exosome

Subcellular Location

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Subcellular Location
Cell membrane
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR000796 Aspartate/other aminotransferaseFamilyFamily
IPR004838 Aminotransferases, class-I, pyridoxal-phosphate-binding siteSiteBinding site
IPR004839 Aminotransferase, class I/classIIDomainDomain
IPR015421 Pyridoxal phosphate-dependent transferase, major domainFamilyHomologous superfamily
IPR015422 Pyridoxal phosphate-dependent transferase, small domainFamilyHomologous superfamily
IPR015424 Pyridoxal phosphate-dependent transferaseFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
618721 OMIMDevelopmental and epileptic encephalopathy 82 (DEE82)A form of epileptic encephalopathy, a heterogeneous group of severe early-onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE82 is an autosomal recessive metabolic encephalopathy characterized by epilepsy from the first year of life, global developmental delay, hypotonia and feeding difficulties apparent soon after birth, and intellectual and motor disabilities. Other features include poor overall growth, progressive microcephaly and biochemical abnormalities, including increased serum lactate and ammonia. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00114 Pyridoxal phosphateDrugbanksmall molecule
DB00128 Aspartic acidDrugbanksmall molecule
DB00142 Glutamic acidDrugbanksmall molecule
DB00151 CysteineDrugbanksmall molecule
DB02783 4'-Deoxy-4'-Acetylyamino-Pyridoxal-5'-PhosphateDrugbanksmall molecule