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Q99K67

- AASS_MOUSE

UniProt

Q99K67 - AASS_MOUSE

Protein

Alpha-aminoadipic semialdehyde synthase, mitochondrial

Gene

Aass

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 106 (01 Oct 2014)
      Sequence version 1 (01 Jun 2001)
      Previous versions | rss
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    Functioni

    Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively.

    Catalytic activityi

    N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP+ + H2O = L-lysine + 2-oxoglutarate + NADPH.
    N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD+ + H2O = L-glutamate + (S)-2-amino-6-oxohexanoate + NADH.

    Pathwayi

    GO - Molecular functioni

    1. saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity Source: UniProtKB
    2. saccharopine dehydrogenase (NADP+, L-lysine-forming) activity Source: UniProtKB-EC

    GO - Biological processi

    1. generation of precursor metabolites and energy Source: UniProtKB
    2. L-lysine catabolic process Source: MGI
    3. L-lysine catabolic process to acetyl-CoA via saccharopine Source: UniProtKB-UniPathway

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Ligandi

    NAD, NADP

    Enzyme and pathway databases

    UniPathwayiUPA00868; UER00835.
    UPA00868; UER00836.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Alpha-aminoadipic semialdehyde synthase, mitochondrial
    Alternative name(s):
    LKR/SDH
    Including the following 2 domains:
    Lysine ketoglutarate reductase (EC:1.5.1.8)
    Short name:
    LKR
    Short name:
    LOR
    Saccharopine dehydrogenase (EC:1.5.1.9)
    Short name:
    SDH
    Gene namesi
    Name:Aass
    Synonyms:Lorsdh
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 6

    Organism-specific databases

    MGIiMGI:1353573. Aass.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrion Source: UniProtKB

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3232MitochondrionAdd
    BLAST
    Chaini33 – 926894Alpha-aminoadipic semialdehyde synthase, mitochondrialPRO_0000001053Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei48 – 481N6-acetyllysine1 Publication
    Modified residuei52 – 521N6-acetyllysine1 Publication
    Modified residuei56 – 561N6-acetyllysine1 Publication
    Modified residuei93 – 931N6-acetyllysine; alternate1 Publication
    Modified residuei93 – 931N6-succinyllysine; alternate1 Publication
    Modified residuei128 – 1281N6-acetyllysine1 Publication
    Modified residuei138 – 1381N6-acetyllysine; alternate1 Publication
    Modified residuei138 – 1381N6-succinyllysine; alternate1 Publication
    Modified residuei274 – 2741N6-succinyllysine1 Publication
    Modified residuei286 – 2861N6-acetyllysine; alternate1 Publication
    Modified residuei286 – 2861N6-succinyllysine; alternate1 Publication
    Modified residuei333 – 3331N6-succinyllysine1 Publication
    Modified residuei458 – 4581N6-acetyllysine; alternate1 Publication
    Modified residuei458 – 4581N6-succinyllysine; alternate1 Publication
    Modified residuei523 – 5231N6-acetyllysine; alternate1 Publication
    Modified residuei523 – 5231N6-succinyllysine; alternate1 Publication
    Modified residuei535 – 5351N6-acetyllysine; alternate1 Publication
    Modified residuei535 – 5351N6-succinyllysine; alternate1 Publication
    Modified residuei584 – 5841N6-acetyllysine; alternate1 Publication
    Modified residuei584 – 5841N6-succinyllysine; alternate1 Publication
    Modified residuei707 – 7071N6-acetyllysine1 Publication
    Modified residuei732 – 7321N6-succinyllysine1 Publication
    Modified residuei739 – 7391N6-acetyllysine1 Publication
    Modified residuei761 – 7611N6-acetyllysine; alternate1 Publication
    Modified residuei761 – 7611N6-succinyllysine; alternate1 Publication
    Modified residuei778 – 7781N6-acetyllysine1 Publication
    Modified residuei780 – 7801N6-acetyllysine1 Publication

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiQ99K67.
    PaxDbiQ99K67.
    PRIDEiQ99K67.

    2D gel databases

    REPRODUCTION-2DPAGEQ99K67.

    PTM databases

    PhosphoSiteiQ99K67.

    Expressioni

    Tissue specificityi

    Both enzymes are highly expressed in kidney and liver, very low expression is seen in heart, brain, spleen, lung, skeletal muscle and testis. SDH was detected only in cortical regions of the kidney.

    Inductioni

    Induced by starvation.

    Gene expression databases

    ArrayExpressiQ99K67.
    BgeeiQ99K67.
    CleanExiMM_AASS.
    GenevestigatoriQ99K67.

    Interactioni

    Subunit structurei

    Homodimer.

    Protein-protein interaction databases

    IntActiQ99K67. 2 interactions.
    MINTiMINT-1840773.

    Structurei

    3D structure databases

    ProteinModelPortaliQ99K67.
    SMRiQ99K67. Positions 30-424, 481-912.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni33 – 455423Lysine-ketoglutarate reductaseAdd
    BLAST
    Regioni477 – 926450Saccharopine dehydrogenaseAdd
    BLAST

    Sequence similaritiesi

    In the N-terminal section; belongs to the AlaDH/PNT family.Curated
    In the C-terminal section; belongs to the saccharopine dehydrogenase family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG1748.
    GeneTreeiENSGT00390000013249.
    HOGENOMiHOG000252920.
    HOVERGENiHBG048688.
    InParanoidiQ99K67.
    KOiK14157.
    OMAiILKQEIR.
    OrthoDBiEOG7F7W7Z.
    PhylomeDBiQ99K67.
    TreeFamiTF105728.

    Family and domain databases

    Gene3Di3.40.50.720. 1 hit.
    InterProiIPR007886. AlaDH/PNT_N.
    IPR007698. AlaDH/PNT_NAD(H)-bd.
    IPR016040. NAD(P)-bd_dom.
    IPR005097. Saccharopine_DH/HSpermid_syn.
    [Graphical view]
    PfamiPF01262. AlaDh_PNT_C. 1 hit.
    PF05222. AlaDh_PNT_N. 1 hit.
    PF03435. Saccharop_dh. 1 hit.
    [Graphical view]
    SMARTiSM01002. AlaDh_PNT_C. 1 hit.
    SM01003. AlaDh_PNT_N. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    Q99K67-1 [UniParc]FASTAAdd to Basket

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    MLRAQRPRLA RLRACLSRGL HHKPVMALRR EDVNAWERRA PLAPKHIKGI    50
    TKLGYKVLIQ PSNRRAIHDK EYVRAGGILQ EDITEACLIL GVKRPPEEKL 100
    MSKKTYAFFS HTIKAQEANM NLLDEVLKQE IRLIDYEKMV DHRGSRIVAF 150
    GQWAGVAGMI NILHGMGLRL LALGHHTPFM HLGMAHNYRN SSQAVQAVRD 200
    AGYEISLGLM PKSIGPLTFV FTGTGNVSKG AQEVFNELPC EYVEPHELRE 250
    VSKTGDLRKV YGTVLSRHHH LVRKTDGVYD PVEYEKYPER YTSRFNTDIA 300
    PYTTCLINGI YWEQNTPRLL TRQDAQSLLV PVKSSVVPVE GCPELPHKLV 350
    AICDISADTG GSIDFMTECT TIERPFCMYD ADQQIIHDSV EGSGILMCSI 400
    DNLPAQLPIE ATEYFGDMLY PYVEEMLLSD ASQPLESQNF SPVVRDAVIT 450
    SNGLLTDKYK YIQKLRESRE RIQFLSMSTK KKVLVLGSGY VSGPVLEYLS 500
    RDNNIEITLG SDMTNQMQQL SKKYNINPVS LTVGKQEAKL QSLVESQDLV 550
    ISLLPYVLHP VVAKACIESR VNMVTASYIT PAMKELEKSV DDAGITVIGE 600
    LGLDPGLDHM LAMETIDTAK ELGATVESYV SYCGGLPAPE HSDNPLRYKF 650
    SWSPVGVLMN IMQPASYLLN GKVVNVTGGV SFLNSVTPMD YFPGLNLEGY 700
    PNRDSIKYAE IYGISSAHTL LRGTLRYKGY SKALNGFVKL GLINREAYPA 750
    LRPEANPLTW KQLLCDLVGI SRSSPCEKLK EVVFTKLGGD NTQLEAAEWL 800
    GLLGDEQVPQ AESIVDAFSK HLVSKLSYGP EEKDMIVMRD SFGIRHPSGH 850
    LENKTIDLVV YGDFNGFSAM AKTVGLPTAM AAKMLLDGEI EAKGLMGPFT 900
    KEIYGPILER IKAEGIVFNT QSTIKL 926
    Length:926
    Mass (Da):102,975
    Last modified:June 1, 2001 - v1
    Checksum:i5B4369C51F7D1D53
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti198 – 1981V → I in CAA12114. (PubMed:10567240)Curated
    Sequence conflicti851 – 8511L → A in CAA12114. (PubMed:10567240)Curated

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AJ224761 mRNA. Translation: CAA12114.1.
    BC005420 mRNA. Translation: AAH05420.1.
    CCDSiCCDS19937.1.
    RefSeqiNP_038958.2. NM_013930.4.
    UniGeneiMm.18651.

    Genome annotation databases

    EnsembliENSMUST00000031707; ENSMUSP00000031707; ENSMUSG00000029695.
    GeneIDi30956.
    KEGGimmu:30956.
    UCSCiuc009bbc.2. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AJ224761 mRNA. Translation: CAA12114.1 .
    BC005420 mRNA. Translation: AAH05420.1 .
    CCDSi CCDS19937.1.
    RefSeqi NP_038958.2. NM_013930.4.
    UniGenei Mm.18651.

    3D structure databases

    ProteinModelPortali Q99K67.
    SMRi Q99K67. Positions 30-424, 481-912.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi Q99K67. 2 interactions.
    MINTi MINT-1840773.

    PTM databases

    PhosphoSitei Q99K67.

    2D gel databases

    REPRODUCTION-2DPAGE Q99K67.

    Proteomic databases

    MaxQBi Q99K67.
    PaxDbi Q99K67.
    PRIDEi Q99K67.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000031707 ; ENSMUSP00000031707 ; ENSMUSG00000029695 .
    GeneIDi 30956.
    KEGGi mmu:30956.
    UCSCi uc009bbc.2. mouse.

    Organism-specific databases

    CTDi 10157.
    MGIi MGI:1353573. Aass.

    Phylogenomic databases

    eggNOGi COG1748.
    GeneTreei ENSGT00390000013249.
    HOGENOMi HOG000252920.
    HOVERGENi HBG048688.
    InParanoidi Q99K67.
    KOi K14157.
    OMAi ILKQEIR.
    OrthoDBi EOG7F7W7Z.
    PhylomeDBi Q99K67.
    TreeFami TF105728.

    Enzyme and pathway databases

    UniPathwayi UPA00868 ; UER00835 .
    UPA00868 ; UER00836 .

    Miscellaneous databases

    NextBioi 307440.
    PROi Q99K67.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q99K67.
    Bgeei Q99K67.
    CleanExi MM_AASS.
    Genevestigatori Q99K67.

    Family and domain databases

    Gene3Di 3.40.50.720. 1 hit.
    InterProi IPR007886. AlaDH/PNT_N.
    IPR007698. AlaDH/PNT_NAD(H)-bd.
    IPR016040. NAD(P)-bd_dom.
    IPR005097. Saccharopine_DH/HSpermid_syn.
    [Graphical view ]
    Pfami PF01262. AlaDh_PNT_C. 1 hit.
    PF05222. AlaDh_PNT_N. 1 hit.
    PF03435. Saccharop_dh. 1 hit.
    [Graphical view ]
    SMARTi SM01002. AlaDh_PNT_C. 1 hit.
    SM01003. AlaDh_PNT_N. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Lysine degradation through the saccharopine pathway in mammals: involvement of both bifunctional and monofunctional lysine-degrading enzymes in mouse."
      Papes F., Kemper E.L., Cord-Neto G., Langone F., Arruda P.
      Biochem. J. 344:555-563(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Strain: C57BL/6J X CBA/J.
      Tissue: Liver.
    2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Mammary tumor.
    3. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
      Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
      Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-93; LYS-138; LYS-274; LYS-286; LYS-333; LYS-458; LYS-523; LYS-535; LYS-584; LYS-732 AND LYS-761, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.
    4. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
      Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
      Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-48; LYS-52; LYS-56; LYS-93; LYS-128; LYS-138; LYS-286; LYS-458; LYS-523; LYS-535; LYS-584; LYS-707; LYS-739; LYS-761; LYS-778 AND LYS-780, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.

    Entry informationi

    Entry nameiAASS_MOUSE
    AccessioniPrimary (citable) accession number: Q99K67
    Secondary accession number(s): Q9Z1I9
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: April 13, 2004
    Last sequence update: June 1, 2001
    Last modified: October 1, 2014
    This is version 106 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Multifunctional enzyme, Reference proteome

    Documents

    1. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
    2. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3