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Protein

Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial

Gene

AADAT

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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

L-kynurenine + 2-oxoglutarate = 4-(2-aminophenyl)-2,4-dioxobutanoate + L-glutamate.
L-2-aminoadipate + 2-oxoglutarate = 2-oxoadipate + L-glutamate.

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

  1. KM=0.9 mM for aminoadipate1 Publication
  2. KM=4.7 mM for kynurenine1 Publication
  3. KM=1.7 mM for methionine1 Publication
  4. KM=1.6 mM for glutamate1 Publication
  5. KM=1.8 mM for tyrosine1 Publication
  6. KM=1.2 mM for 2-oxoglutarate1 Publication
  7. KM=1.5 mM for 2-oxocaproic acid1 Publication
  8. KM=1.8 mM for phenylpyruvate1 Publication
  9. 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

    Pathwayi: L-lysine degradation via saccharopine pathway

    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:
    1. Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS)
    2. Alpha-aminoadipic semialdehyde synthase, mitochondrial (AASS)
    3. no protein annotated in this organism
    4. Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial (AADAT)
    5. no protein annotated in this organism
    6. Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial (DLST), Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex (sucB)
    This subpathway is part of the pathway L-lysine degradation via saccharopine pathway, which is itself part of Amino-acid degradation.
    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 keyPosition(s)DescriptionActionsGraphical viewLength
    Binding sitei20Substrate1
    Binding sitei74Substrate1
    Binding sitei142Substrate1
    Binding sitei202Substrate1
    Binding sitei399Substrate1

    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

    GO - Biological processi

    Keywordsi

    Molecular functionAminotransferase, Transferase
    LigandPyridoxal phosphate

    Enzyme and pathway databases

    BioCyciMetaCyc:HS03239-MONOMER
    BRENDAi2.6.1.39 2681
    2.6.1.7 2681
    ReactomeiR-HSA-71064 Lysine catabolism
    R-HSA-71240 Tryptophan catabolism
    SABIO-RKiQ8N5Z0
    UniPathwayi
    UPA00868;UER00838

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
    Short name:
    KAT/AadAT
    Alternative name(s):
    2-aminoadipate aminotransferase
    2-aminoadipate transaminase (EC:2.6.1.39)
    Alpha-aminoadipate aminotransferase
    Short name:
    AadAT
    Kynurenine aminotransferase II
    Kynurenine--oxoglutarate aminotransferase II
    Kynurenine--oxoglutarate transaminase 2 (EC:2.6.1.7)
    Kynurenine--oxoglutarate transaminase II
    Gene namesi
    Name:AADAT
    Synonyms:KAT2
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 4

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000109576.13
    HGNCiHGNC:17929 AADAT
    MIMi611754 gene
    neXtProtiNX_Q8N5Z0

    Subcellular locationi

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    DisGeNETi51166
    OpenTargetsiENSG00000109576
    PharmGKBiPA24364

    Chemistry databases

    ChEMBLiCHEMBL2046259
    DrugBankiDB00142 L-Glutamic Acid
    DB00114 Pyridoxal Phosphate

    Polymorphism and mutation databases

    BioMutaiAADAT
    DMDMi46395904

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Transit peptidei1 – 29MitochondrionSequence analysisAdd BLAST29
    ChainiPRO_000002060230 – 425Kynurenine/alpha-aminoadipate aminotransferase, mitochondrialAdd BLAST396

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei69N6-acetyllysineBy similarity1
    Modified residuei179N6-acetyllysineBy similarity1
    Modified residuei263N6-(pyridoxal phosphate)lysine; alternate1
    Modified residuei263N6-acetyllysine; alternateBy similarity1
    Modified residuei263N6-succinyllysine; alternateBy similarity1
    Modified residuei339N6-acetyllysine; alternateBy similarity1
    Modified residuei339N6-succinyllysine; alternateBy similarity1
    Modified residuei367N6-acetyllysine; alternateBy similarity1
    Modified residuei367N6-succinyllysine; alternateBy similarity1
    Modified residuei422N6-acetyllysineBy similarity1

    Keywords - PTMi

    Acetylation

    Proteomic databases

    EPDiQ8N5Z0
    MaxQBiQ8N5Z0
    PaxDbiQ8N5Z0
    PeptideAtlasiQ8N5Z0
    PRIDEiQ8N5Z0
    ProteomicsDBi72114
    72115 [Q8N5Z0-2]

    PTM databases

    iPTMnetiQ8N5Z0
    PhosphoSitePlusiQ8N5Z0

    Expressioni

    Tissue specificityi

    Higher expression in the liver. Also found in heart, brain, kidney, pancreas, prostate, testis and ovary.

    Gene expression databases

    BgeeiENSG00000109576 Expressed in 148 organ(s), highest expression level in right lobe of liver
    CleanExiHS_AADAT
    ExpressionAtlasiQ8N5Z0 baseline and differential
    GenevisibleiQ8N5Z0 HS

    Organism-specific databases

    HPAiHPA037502

    Interactioni

    Subunit structurei

    Homodimer.3 Publications

    GO - Molecular functioni

    Protein-protein interaction databases

    BioGridi119346, 2 interactors
    STRINGi9606.ENSP00000226840

    Chemistry databases

    BindingDBiQ8N5Z0

    Structurei

    Secondary structure

    1425
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    ProteinModelPortaliQ8N5Z0
    SMRiQ8N5Z0
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiQ8N5Z0

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiKOG0634 Eukaryota
    COG1167 LUCA
    GeneTreeiENSGT00390000004594
    HOGENOMiHOG000223057
    HOVERGENiHBG050429
    InParanoidiQ8N5Z0
    KOiK00825
    OMAiMRLNFTY
    OrthoDBiEOG091G0B8J
    PhylomeDBiQ8N5Z0
    TreeFamiTF328598

    Family and domain databases

    Gene3Di3.40.640.10, 1 hit
    InterProiView protein in InterPro
    IPR004839 Aminotransferase_I/II
    IPR015424 PyrdxlP-dep_Trfase
    IPR015421 PyrdxlP-dep_Trfase_major
    PfamiView protein in Pfam
    PF00155 Aminotran_1_2, 1 hit
    SUPFAMiSSF53383 SSF53383, 1 hit

    Sequences (2+)i

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

    This entry has 2 described isoforms and 3 potential isoforms that are computationally mapped.iShow all

    Isoform 1 (identifier: Q8N5Z0-1) [UniParc]FASTAAdd to basket

    This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide
            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.
    Length:425
    Mass (Da):47,352
    Last modified:April 13, 2004 - v2
    Checksum:i448CCAAB2173A7BA
    GO
    Isoform 2 (identifier: Q8N5Z0-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         23-23: T → SEKRA

    Show »
    Length:429
    Mass (Da):47,822
    Checksum:i0C5A6E51F1BD6F53
    GO

    Computationally mapped potential isoform sequencesi

    There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    Gene namesLengthAnnotation
    D6RFY7D6RFY7_HUMAN
    Kynurenine/alpha-aminoadipate amino...
    AADAT
    209Annotation score:
    D6REB9D6REB9_HUMAN
    Kynurenine/alpha-aminoadipate amino...
    AADAT
    220Annotation score:
    D6RC56D6RC56_HUMAN
    Kynurenine/alpha-aminoadipate amino...
    AADAT
    180Annotation score:

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Sequence conflicti103P → Q in AAH31068 (PubMed:15489334).Curated1
    Sequence conflicti380L → S in BAG51596 (PubMed:14702039).Curated1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_061005243V → I. Corresponds to variant dbSNP:rs56350236Ensembl.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Alternative sequenceiVSP_00987423T → SEKRA in isoform 2. 1 Publication1

    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
    CCDSiCCDS3814.1 [Q8N5Z0-1]
    CCDS75209.1 [Q8N5Z0-2]
    RefSeqiNP_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]
    UniGeneiHs.529735

    Genome annotation databases

    EnsembliENST00000337664; ENSP00000336808; ENSG00000109576 [Q8N5Z0-1]
    ENST00000353187; ENSP00000226840; ENSG00000109576 [Q8N5Z0-1]
    ENST00000509167; ENSP00000423190; ENSG00000109576 [Q8N5Z0-2]
    ENST00000515480; ENSP00000423341; ENSG00000109576 [Q8N5Z0-1]
    GeneIDi51166
    KEGGihsa:51166
    UCSCiuc003isr.4 human [Q8N5Z0-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    Similar proteinsi

    Entry informationi

    Entry nameiAADAT_HUMAN
    AccessioniPrimary (citable) accession number: Q8N5Z0
    Secondary accession number(s): B3KP84, Q9UL02
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 13, 2004
    Last sequence update: April 13, 2004
    Last modified: September 12, 2018
    This is version 147 of the entry and version 2 of the sequence. See complete history.
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny 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, Complete proteome, Reference proteome
    UniProt is an ELIXIR core data resource
    Main funding by: National Institutes of Health

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