UniProtKB - Q8N5Z0 (AADAT_HUMAN)
Protein
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
Gene
AADAT
Organism
Homo sapiens (Human)
Status
Functioni
Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro).1 Publication
Catalytic activityi
Cofactori
pyridoxal 5'-phosphate3 Publications
Activity regulationi
Kynurenine transaminase activity is competitively inhibited by aminoadipate, asparagine, glutamate, histidine, cysteine, lysine, 3-hydroxy-kynurenine and phenylalanine.1 Publication
Kineticsi
- KM=0.9 mM for aminoadipate1 Publication
- KM=4.7 mM for kynurenine1 Publication
- KM=1.7 mM for methionine1 Publication
- KM=1.6 mM for glutamate1 Publication
- KM=1.8 mM for tyrosine1 Publication
- KM=1.2 mM for 2-oxoglutarate1 Publication
- KM=1.5 mM for 2-oxocaproic acid1 Publication
- KM=1.8 mM for phenylpyruvate1 Publication
- KM=1.4 mM for ino-3-pyruvate1 Publication
pH dependencei
Optimum pH is 7-9.1 Publication
Temperature dependencei
Optimum temperature is 50 degrees Celsius.1 Publication
: L-lysine degradation via saccharopine pathway Pathwayi
This protein is involved in step 4 of the subpathway that synthesizes glutaryl-CoA from L-lysine.Proteins known to be involved in the 6 steps of the subpathway in this organism are:
- Lysine ketoglutarate reductase (AASS), Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS), Lysine ketoglutarate reductase (AASS)
- Lysine ketoglutarate reductase (AASS), Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS), Lysine ketoglutarate reductase (AASS)
- no protein annotated in this organism
- Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial (AADAT)
- no protein annotated in this organism
- Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (DLST), Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (DLST), Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (DLST), Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (E2k)
View all proteins of this organism that are known to be involved in the subpathway that synthesizes glutaryl-CoA from L-lysine, the pathway L-lysine degradation via saccharopine pathway and in Amino-acid degradation.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 20 | Substrate | 1 | |
Binding sitei | 74 | Substrate | 1 | |
Binding sitei | 142 | Substrate | 1 | |
Binding sitei | 202 | Substrate | 1 | |
Binding sitei | 399 | Substrate | 1 |
GO - Molecular functioni
- 2-aminoadipate transaminase activity Source: UniProtKB
- kynurenine-oxoglutarate transaminase activity Source: UniProtKB
- protein homodimerization activity Source: UniProtKB
- pyridoxal phosphate binding Source: InterPro
- transaminase activity Source: GO_Central
GO - Biological processi
- 2-oxoglutarate metabolic process Source: UniProtKB
- alpha-amino acid metabolic process Source: GO_Central
- biosynthetic process Source: InterPro
- glutamate metabolic process Source: UniProtKB
- kynurenine metabolic process Source: UniProtKB
- L-lysine catabolic process to acetyl-CoA via saccharopine Source: UniProtKB-UniPathway
- lysine catabolic process Source: Reactome
- tryptophan catabolic process Source: Reactome
Keywordsi
Molecular function | Aminotransferase, Transferase |
Ligand | Pyridoxal phosphate |
Enzyme and pathway databases
BioCyci | MetaCyc:HS03239-MONOMER |
BRENDAi | 2.6.1.39, 2681 2.6.1.7, 2681 |
PathwayCommonsi | Q8N5Z0 |
Reactomei | R-HSA-71064, Lysine catabolism R-HSA-71240, Tryptophan catabolism |
SABIO-RKi | Q8N5Z0 |
UniPathwayi | UPA00868;UER00838 |
Names & Taxonomyi
Protein namesi | Recommended name: Kynurenine/alpha-aminoadipate aminotransferase, mitochondrialShort name: KAT/AadAT Alternative name(s): |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:17929, AADAT |
MIMi | 611754, gene |
neXtProti | NX_Q8N5Z0 |
VEuPathDBi | HostDB:ENSG00000109576.13 |
Subcellular locationi
Mitochondrion
- Mitochondrion Curated
Mitochondrion
- mitochondrial matrix Source: Reactome
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Organism-specific databases
DisGeNETi | 51166 |
OpenTargetsi | ENSG00000109576 |
PharmGKBi | PA24364 |
Miscellaneous databases
Pharosi | Q8N5Z0, Tchem |
Chemistry databases
ChEMBLi | CHEMBL2046259 |
DrugBanki | DB00142, Glutamic acid DB00114, Pyridoxal phosphate |
Genetic variation databases
BioMutai | AADAT |
DMDMi | 46395904 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 29 | MitochondrionSequence analysisAdd BLAST | 29 | |
ChainiPRO_0000020602 | 30 – 425 | Kynurenine/alpha-aminoadipate aminotransferase, mitochondrialAdd BLAST | 396 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 69 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 179 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 263 | N6-(pyridoxal phosphate)lysine; alternate | 1 | |
Modified residuei | 263 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 263 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 339 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 339 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 367 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 367 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 422 | N6-acetyllysineBy similarity | 1 |
Keywords - PTMi
AcetylationProteomic databases
EPDi | Q8N5Z0 |
jPOSTi | Q8N5Z0 |
MassIVEi | Q8N5Z0 |
MaxQBi | Q8N5Z0 |
PaxDbi | Q8N5Z0 |
PeptideAtlasi | Q8N5Z0 |
PRIDEi | Q8N5Z0 |
ProteomicsDBi | 72114 [Q8N5Z0-1] 72115 [Q8N5Z0-2] |
PTM databases
iPTMneti | Q8N5Z0 |
PhosphoSitePlusi | Q8N5Z0 |
Expressioni
Tissue specificityi
Higher expression in the liver. Also found in heart, brain, kidney, pancreas, prostate, testis and ovary.
Gene expression databases
Bgeei | ENSG00000109576, Expressed in liver and 165 other tissues |
ExpressionAtlasi | Q8N5Z0, baseline and differential |
Genevisiblei | Q8N5Z0, HS |
Organism-specific databases
HPAi | ENSG00000109576, Tissue enriched (liver) |
Interactioni
Subunit structurei
Homodimer.
3 PublicationsGO - Molecular functioni
- protein homodimerization activity Source: UniProtKB
Protein-protein interaction databases
BioGRIDi | 119346, 21 interactors |
IntActi | Q8N5Z0, 1 interactor |
STRINGi | 9606.ENSP00000423190 |
Chemistry databases
BindingDBi | Q8N5Z0 |
Miscellaneous databases
RNActi | Q8N5Z0, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | Q8N5Z0 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | Q8N5Z0 |
Family & Domainsi
Sequence similaritiesi
Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG0634, Eukaryota |
GeneTreei | ENSGT00390000004594 |
HOGENOMi | CLU_017584_0_6_1 |
InParanoidi | Q8N5Z0 |
OMAi | MRLNFTY |
OrthoDBi | 1241781at2759 |
PhylomeDBi | Q8N5Z0 |
TreeFami | TF328598 |
Family and domain databases
Gene3Di | 3.40.640.10, 1 hit |
InterProi | View protein in InterPro IPR004839, Aminotransferase_I/II IPR015424, PyrdxlP-dep_Trfase IPR015421, PyrdxlP-dep_Trfase_major |
Pfami | View protein in Pfam PF00155, Aminotran_1_2, 1 hit |
SUPFAMi | SSF53383, SSF53383, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: Q8N5Z0-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MNYARFITAA SAARNPSPIR TMTDILSRGP KSMISLAGGL PNPNMFPFKT
60 70 80 90 100
AVITVENGKT IQFGEEMMKR ALQYSPSAGI PELLSWLKQL QIKLHNPPTI
110 120 130 140 150
HYPPSQGQMD LCVTSGSQQG LCKVFEMIIN PGDNVLLDEP AYSGTLQSLH
160 170 180 190 200
PLGCNIINVA SDESGIVPDS LRDILSRWKP EDAKNPQKNT PKFLYTVPNG
210 220 230 240 250
NNPTGNSLTS ERKKEIYELA RKYDFLIIED DPYYFLQFNK FRVPTFLSMD
260 270 280 290 300
VDGRVIRADS FSKIISSGLR IGFLTGPKPL IERVILHIQV STLHPSTFNQ
310 320 330 340 350
LMISQLLHEW GEEGFMAHVD RVIDFYSNQK DAILAAADKW LTGLAEWHVP
360 370 380 390 400
AAGMFLWIKV KGINDVKELI EEKAVKMGVL MLPGNAFYVD SSAPSPYLRA
410 420
SFSSASPEQM DVAFQVLAQL IKESL
Note: May be due to a competing donor splice site.
Computationally mapped potential isoform sequencesi
There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketD6REB9 | D6REB9_HUMAN | Kynurenine/alpha-aminoadipate amino... | AADAT | 220 | Annotation score: | ||
D6RFY7 | D6RFY7_HUMAN | Kynurenine/alpha-aminoadipate amino... | AADAT | 209 | Annotation score: | ||
D6RC56 | D6RC56_HUMAN | Kynurenine/alpha-aminoadipate amino... | AADAT | 180 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 103 | P → Q in AAH31068 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 380 | L → S in BAG51596 (PubMed:14702039).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_061005 | 243 | V → I. Corresponds to variant dbSNP:rs56350236Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_009874 | 23 | T → SEKRA in isoform 2. 1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF097994 mRNA Translation: AAF04623.1 AF481738 mRNA Translation: AAM09683.1 AK055952 mRNA Translation: BAG51596.1 BC031068 mRNA Translation: AAH31068.1 |
CCDSi | CCDS3814.1 [Q8N5Z0-1] CCDS75209.1 [Q8N5Z0-2] |
RefSeqi | NP_001273611.1, NM_001286682.1 [Q8N5Z0-2] NP_001273612.1, NM_001286683.1 [Q8N5Z0-1] NP_057312.1, NM_016228.3 [Q8N5Z0-1] NP_872603.1, NM_182662.1 [Q8N5Z0-1] |
Genome annotation databases
Ensembli | ENST00000337664; ENSP00000336808; ENSG00000109576 [Q8N5Z0-1] ENST00000353187; ENSP00000226840; ENSG00000109576 [Q8N5Z0-1] ENST00000509167; ENSP00000423190; ENSG00000109576 [Q8N5Z0-2] ENST00000515480; ENSP00000423341; ENSG00000109576 [Q8N5Z0-1] |
GeneIDi | 51166 |
KEGGi | hsa:51166 |
UCSCi | uc003isr.4, human [Q8N5Z0-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF097994 mRNA Translation: AAF04623.1 AF481738 mRNA Translation: AAM09683.1 AK055952 mRNA Translation: BAG51596.1 BC031068 mRNA Translation: AAH31068.1 |
CCDSi | CCDS3814.1 [Q8N5Z0-1] CCDS75209.1 [Q8N5Z0-2] |
RefSeqi | NP_001273611.1, NM_001286682.1 [Q8N5Z0-2] NP_001273612.1, NM_001286683.1 [Q8N5Z0-1] NP_057312.1, NM_016228.3 [Q8N5Z0-1] NP_872603.1, NM_182662.1 [Q8N5Z0-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
2QLR | X-ray | 2.30 | A/B/C/D | 1-425 | [»] | |
2R2N | X-ray | 1.95 | A/B/C/D | 1-425 | [»] | |
2VGZ | X-ray | 2.30 | A/B | 2-425 | [»] | |
2XH1 | X-ray | 2.10 | A/B | 1-425 | [»] | |
3DC1 | X-ray | 2.50 | A/B/C/D | 1-425 | [»] | |
3UE8 | X-ray | 3.22 | A/B | 1-425 | [»] | |
4GDY | X-ray | 2.89 | A/B | 1-425 | [»] | |
4GE4 | X-ray | 2.41 | A/B | 1-425 | [»] | |
4GE7 | X-ray | 2.10 | A/B | 1-425 | [»] | |
4GE9 | X-ray | 2.43 | A/B/C/D | 1-425 | [»] | |
4GEB | X-ray | 2.15 | A/B | 1-425 | [»] | |
5EFS | X-ray | 1.83 | A | 1-425 | [»] | |
5EUN | X-ray | 1.82 | A | 1-425 | [»] | |
5TF5 | X-ray | 1.81 | A/B | 1-425 | [»] | |
6D0A | X-ray | 1.47 | A | 1-425 | [»] | |
6T8P | X-ray | 2.02 | A/B | 1-425 | [»] | |
6T8Q | X-ray | 2.51 | A | 1-425 | [»] | |
SMRi | Q8N5Z0 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 119346, 21 interactors |
IntActi | Q8N5Z0, 1 interactor |
STRINGi | 9606.ENSP00000423190 |
Chemistry databases
BindingDBi | Q8N5Z0 |
ChEMBLi | CHEMBL2046259 |
DrugBanki | DB00142, Glutamic acid DB00114, Pyridoxal phosphate |
PTM databases
iPTMneti | Q8N5Z0 |
PhosphoSitePlusi | Q8N5Z0 |
Genetic variation databases
BioMutai | AADAT |
DMDMi | 46395904 |
Proteomic databases
EPDi | Q8N5Z0 |
jPOSTi | Q8N5Z0 |
MassIVEi | Q8N5Z0 |
MaxQBi | Q8N5Z0 |
PaxDbi | Q8N5Z0 |
PeptideAtlasi | Q8N5Z0 |
PRIDEi | Q8N5Z0 |
ProteomicsDBi | 72114 [Q8N5Z0-1] 72115 [Q8N5Z0-2] |
Protocols and materials databases
Antibodypediai | 17143, 322 antibodies |
DNASUi | 51166 |
Genome annotation databases
Ensembli | ENST00000337664; ENSP00000336808; ENSG00000109576 [Q8N5Z0-1] ENST00000353187; ENSP00000226840; ENSG00000109576 [Q8N5Z0-1] ENST00000509167; ENSP00000423190; ENSG00000109576 [Q8N5Z0-2] ENST00000515480; ENSP00000423341; ENSG00000109576 [Q8N5Z0-1] |
GeneIDi | 51166 |
KEGGi | hsa:51166 |
UCSCi | uc003isr.4, human [Q8N5Z0-1] |
Organism-specific databases
CTDi | 51166 |
DisGeNETi | 51166 |
GeneCardsi | AADAT |
HGNCi | HGNC:17929, AADAT |
HPAi | ENSG00000109576, Tissue enriched (liver) |
MIMi | 611754, gene |
neXtProti | NX_Q8N5Z0 |
OpenTargetsi | ENSG00000109576 |
PharmGKBi | PA24364 |
VEuPathDBi | HostDB:ENSG00000109576.13 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0634, Eukaryota |
GeneTreei | ENSGT00390000004594 |
HOGENOMi | CLU_017584_0_6_1 |
InParanoidi | Q8N5Z0 |
OMAi | MRLNFTY |
OrthoDBi | 1241781at2759 |
PhylomeDBi | Q8N5Z0 |
TreeFami | TF328598 |
Enzyme and pathway databases
UniPathwayi | UPA00868;UER00838 |
BioCyci | MetaCyc:HS03239-MONOMER |
BRENDAi | 2.6.1.39, 2681 2.6.1.7, 2681 |
PathwayCommonsi | Q8N5Z0 |
Reactomei | R-HSA-71064, Lysine catabolism R-HSA-71240, Tryptophan catabolism |
SABIO-RKi | Q8N5Z0 |
Miscellaneous databases
BioGRID-ORCSi | 51166, 15 hits in 987 CRISPR screens |
ChiTaRSi | AADAT, human |
EvolutionaryTracei | Q8N5Z0 |
GenomeRNAii | 51166 |
Pharosi | Q8N5Z0, Tchem |
PROi | PR:Q8N5Z0 |
RNActi | Q8N5Z0, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000109576, Expressed in liver and 165 other tissues |
ExpressionAtlasi | Q8N5Z0, baseline and differential |
Genevisiblei | Q8N5Z0, HS |
Family and domain databases
Gene3Di | 3.40.640.10, 1 hit |
InterProi | View protein in InterPro IPR004839, Aminotransferase_I/II IPR015424, PyrdxlP-dep_Trfase IPR015421, PyrdxlP-dep_Trfase_major |
Pfami | View protein in Pfam PF00155, Aminotran_1_2, 1 hit |
SUPFAMi | SSF53383, SSF53383, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | AADAT_HUMAN | |
Accessioni | Q8N5Z0Primary (citable) accession number: Q8N5Z0 Secondary accession number(s): B3KP84, Q9UL02 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | April 13, 2004 |
Last sequence update: | April 13, 2004 | |
Last modified: | April 7, 2021 | |
This is version 162 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Reference proteomeDocuments
- Human chromosome 4
Human chromosome 4: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families