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P53597

- SUCA_HUMAN

UniProt

P53597 - SUCA_HUMAN

Protein

Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial

Gene

SUCLG1

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 149 (01 Oct 2014)
      Sequence version 4 (10 Feb 2009)
      Previous versions | rss
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    Functioni

    Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity By similarity.By similarity

    Catalytic activityi

    GTP + succinate + CoA = GDP + phosphate + succinyl-CoA.
    ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.

    Pathwayi

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Active sitei299 – 2991Tele-phosphohistidine intermediateBy similarity

    GO - Molecular functioni

    1. ATP citrate synthase activity Source: InterPro
    2. cofactor binding Source: InterPro
    3. GDP binding Source: Ensembl
    4. GTP binding Source: UniProtKB-KW
    5. poly(A) RNA binding Source: UniProtKB
    6. succinate-CoA ligase (ADP-forming) activity Source: UniProtKB-EC
    7. succinate-CoA ligase (GDP-forming) activity Source: UniProtKB-EC

    GO - Biological processi

    1. cellular metabolic process Source: Reactome
    2. small molecule metabolic process Source: Reactome
    3. succinate metabolic process Source: Ensembl
    4. succinyl-CoA metabolic process Source: Ensembl
    5. tricarboxylic acid cycle Source: Reactome

    Keywords - Molecular functioni

    Ligase

    Keywords - Biological processi

    Tricarboxylic acid cycle

    Keywords - Ligandi

    GTP-binding, Nucleotide-binding

    Enzyme and pathway databases

    BioCyciMetaCyc:HS08877-MONOMER.
    ReactomeiREACT_1785. Citric acid cycle (TCA cycle).
    UniPathwayiUPA00223.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (EC:6.2.1.4, EC:6.2.1.5)
    Alternative name(s):
    Succinyl-CoA synthetase subunit alpha
    Short name:
    SCS-alpha
    Gene namesi
    Name:SUCLG1
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 2

    Organism-specific databases

    HGNCiHGNC:11449. SUCLG1.

    Subcellular locationi

    GO - Cellular componenti

    1. extracellular vesicular exosome Source: UniProt
    2. mitochondrial inner membrane Source: Ensembl
    3. mitochondrial matrix Source: Reactome
    4. mitochondrion Source: ProtInc
    5. succinate-CoA ligase complex (GDP-forming) Source: Ensembl

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Involvement in diseasei

    Mitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM:245400]: A severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid.4 Publications
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti14 – 141M → L in MTDPS9; with progressive liver disease and recurrent hepatic failure. 1 Publication
    VAR_065120
    Natural varianti85 – 851G → A in MTDPS9. 1 Publication
    VAR_065157
    Natural varianti170 – 1701P → R in MTDPS9. 1 Publication
    VAR_065121

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi245400. phenotype.
    Orphaneti17. Fatal infantile lactic acidosis with methylmalonic aciduria.
    PharmGKBiPA36246.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 4040MitochondrionBy similarityAdd
    BLAST
    Chaini41 – 346306Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialPRO_0000033340Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei54 – 541N6-acetyllysine1 Publication
    Modified residuei57 – 571N6-acetyllysine; alternateBy similarity
    Modified residuei57 – 571N6-succinyllysine; alternateBy similarity
    Modified residuei66 – 661N6-acetyllysine; alternateBy similarity
    Modified residuei66 – 661N6-succinyllysine; alternateBy similarity
    Modified residuei81 – 811N6-acetyllysineBy similarity
    Modified residuei105 – 1051N6-acetyllysineBy similarity
    Cross-linki280 – 280Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
    Modified residuei338 – 3381N6-succinyllysineBy similarity

    Keywords - PTMi

    Acetylation, Isopeptide bond, Ubl conjugation

    Proteomic databases

    MaxQBiP53597.
    PaxDbiP53597.
    PRIDEiP53597.

    2D gel databases

    UCD-2DPAGEP53597.

    PTM databases

    PhosphoSiteiP53597.

    Expressioni

    Gene expression databases

    ArrayExpressiP53597.
    BgeeiP53597.
    CleanExiHS_SUCLG1.
    GenevestigatoriP53597.

    Organism-specific databases

    HPAiHPA036683.

    Interactioni

    Subunit structurei

    Heterodimer of an alpha and a beta subunit.

    Protein-protein interaction databases

    BioGridi114330. 13 interactions.
    IntActiP53597. 1 interaction.
    MINTiMINT-137948.
    STRINGi9606.ENSP00000377446.

    Structurei

    3D structure databases

    ProteinModelPortaliP53597.
    SMRiP53597. Positions 41-346.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0074.
    HOGENOMiHOG000239685.
    HOVERGENiHBG000957.
    InParanoidiP53597.
    KOiK01899.
    OMAiQNGVRHC.
    OrthoDBiEOG74BJSG.
    PhylomeDBiP53597.
    TreeFamiTF300666.

    Family and domain databases

    Gene3Di3.40.50.261. 1 hit.
    3.40.50.720. 1 hit.
    InterProiIPR017440. Cit_synth/succinyl-CoA_lig_AS.
    IPR003781. CoA-bd.
    IPR005810. CoA_lig_alpha.
    IPR005811. CoA_ligase.
    IPR016040. NAD(P)-bd_dom.
    IPR016102. Succinyl-CoA_synth-like.
    [Graphical view]
    PfamiPF02629. CoA_binding. 1 hit.
    PF00549. Ligase_CoA. 1 hit.
    [Graphical view]
    PIRSFiPIRSF001553. SucCS_alpha. 1 hit.
    PRINTSiPR01798. SCOASYNTHASE.
    SMARTiSM00881. CoA_binding. 1 hit.
    [Graphical view]
    SUPFAMiSSF52210. SSF52210. 1 hit.
    TIGRFAMsiTIGR01019. sucCoAalpha. 1 hit.
    PROSITEiPS01216. SUCCINYL_COA_LIG_1. 1 hit.
    PS00399. SUCCINYL_COA_LIG_2. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    P53597-1 [UniParc]FASTAAdd to Basket

    « Hide

    MTATLAAAAD IATMVSGSSG LAAARLLSRS FLLPQNGIRH CSYTASRQHL    50
    YVDKNTKIIC QGFTGKQGTF HSQQALEYGT KLVGGTTPGK GGQTHLGLPV 100
    FNTVKEAKEQ TGATASVIYV PPPFAAAAIN EAIEAEIPLV VCITEGIPQQ 150
    DMVRVKHKLL RQEKTRLIGP NCPGVINPGE CKIGIMPGHI HKKGRIGIVS 200
    RSGTLTYEAV HQTTQVGLGQ SLCVGIGGDP FNGTDFIDCL EIFLNDSATE 250
    GIILIGEIGG NAEENAAEFL KQHNSGPNSK PVVSFIAGLT APPGRRMGHA 300
    GAIIAGGKGG AKEKISALQS AGVVVSMSPA QLGTTIYKEF EKRKML 346
    Length:346
    Mass (Da):36,250
    Last modified:February 10, 2009 - v4
    Checksum:i76EF06F323CD5188
    GO

    Sequence cautioni

    The sequence AAD17940.2 differs from that shown. Reason: Erroneous initiation.
    The sequence AAH00504.1 differs from that shown. Reason: Erroneous initiation.
    The sequence CAA92426.1 differs from that shown. Reason: Erroneous initiation.

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti19 – 191S → N in CAA92426. (PubMed:9128182)Curated
    Sequence conflicti34 – 341P → Q in CAA92426. (PubMed:9128182)Curated
    Sequence conflicti39 – 391R → Q in CAA92426. (PubMed:9128182)Curated
    Sequence conflicti87 – 871T → L in AAD17940. 1 PublicationCurated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti14 – 141M → L in MTDPS9; with progressive liver disease and recurrent hepatic failure. 1 Publication
    VAR_065120
    Natural varianti85 – 851G → A in MTDPS9. 1 Publication
    VAR_065157
    Natural varianti170 – 1701P → R in MTDPS9. 1 Publication
    VAR_065121

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AC096770 Genomic DNA. No translation available.
    BC000504 mRNA. Translation: AAH00504.1. Different initiation.
    AF104921 mRNA. Translation: AAD17940.2. Different initiation.
    Z68204 mRNA. Translation: CAA92426.1. Different initiation.
    CCDSiCCDS1967.2.
    RefSeqiNP_003840.2. NM_003849.3.
    UniGeneiHs.270428.

    Genome annotation databases

    EnsembliENST00000393868; ENSP00000377446; ENSG00000163541.
    GeneIDi8802.
    KEGGihsa:8802.
    UCSCiuc002son.3. human.

    Polymorphism databases

    DMDMi223634731.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AC096770 Genomic DNA. No translation available.
    BC000504 mRNA. Translation: AAH00504.1 . Different initiation.
    AF104921 mRNA. Translation: AAD17940.2 . Different initiation.
    Z68204 mRNA. Translation: CAA92426.1 . Different initiation.
    CCDSi CCDS1967.2.
    RefSeqi NP_003840.2. NM_003849.3.
    UniGenei Hs.270428.

    3D structure databases

    ProteinModelPortali P53597.
    SMRi P53597. Positions 41-346.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 114330. 13 interactions.
    IntActi P53597. 1 interaction.
    MINTi MINT-137948.
    STRINGi 9606.ENSP00000377446.

    Chemistry

    DrugBanki DB00139. Succinic acid.

    PTM databases

    PhosphoSitei P53597.

    Polymorphism databases

    DMDMi 223634731.

    2D gel databases

    UCD-2DPAGE P53597.

    Proteomic databases

    MaxQBi P53597.
    PaxDbi P53597.
    PRIDEi P53597.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000393868 ; ENSP00000377446 ; ENSG00000163541 .
    GeneIDi 8802.
    KEGGi hsa:8802.
    UCSCi uc002son.3. human.

    Organism-specific databases

    CTDi 8802.
    GeneCardsi GC02M084562.
    HGNCi HGNC:11449. SUCLG1.
    HPAi HPA036683.
    MIMi 245400. phenotype.
    611224. gene.
    neXtProti NX_P53597.
    Orphaneti 17. Fatal infantile lactic acidosis with methylmalonic aciduria.
    PharmGKBi PA36246.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0074.
    HOGENOMi HOG000239685.
    HOVERGENi HBG000957.
    InParanoidi P53597.
    KOi K01899.
    OMAi QNGVRHC.
    OrthoDBi EOG74BJSG.
    PhylomeDBi P53597.
    TreeFami TF300666.

    Enzyme and pathway databases

    UniPathwayi UPA00223 .
    BioCyci MetaCyc:HS08877-MONOMER.
    Reactomei REACT_1785. Citric acid cycle (TCA cycle).

    Miscellaneous databases

    ChiTaRSi SUCLG1. human.
    GeneWikii SUCLG1.
    GenomeRNAii 8802.
    NextBioi 33016.
    PROi P53597.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P53597.
    Bgeei P53597.
    CleanExi HS_SUCLG1.
    Genevestigatori P53597.

    Family and domain databases

    Gene3Di 3.40.50.261. 1 hit.
    3.40.50.720. 1 hit.
    InterProi IPR017440. Cit_synth/succinyl-CoA_lig_AS.
    IPR003781. CoA-bd.
    IPR005810. CoA_lig_alpha.
    IPR005811. CoA_ligase.
    IPR016040. NAD(P)-bd_dom.
    IPR016102. Succinyl-CoA_synth-like.
    [Graphical view ]
    Pfami PF02629. CoA_binding. 1 hit.
    PF00549. Ligase_CoA. 1 hit.
    [Graphical view ]
    PIRSFi PIRSF001553. SucCS_alpha. 1 hit.
    PRINTSi PR01798. SCOASYNTHASE.
    SMARTi SM00881. CoA_binding. 1 hit.
    [Graphical view ]
    SUPFAMi SSF52210. SSF52210. 1 hit.
    TIGRFAMsi TIGR01019. sucCoAalpha. 1 hit.
    PROSITEi PS01216. SUCCINYL_COA_LIG_1. 1 hit.
    PS00399. SUCCINYL_COA_LIG_2. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
      Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H.
      , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.
      Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    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] OF 3-346.
      Tissue: Lung.
    3. "Sequence of the alpha subunit of succinyl-CoA synthetase in human."
      Tews K.N., Mehus J.G., Johnson J.D., Milavetz B.I., Lambeth D.O.
      Submitted (JUL-2000) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 4-346.
    4. "The molecular basis for cross-reaction anti-dystrophin antibody with alpha-actinin."
      James M., Man N.T., Edwards Y.H., Morris G.E.
      Biochim. Biophys. Acta 1360:169-176(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 12-145.
      Tissue: Brain and Skeletal muscle.
    5. "Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion."
      Ostergaard E., Christensen E., Kristensen E., Mogensen B., Duno M., Shoubridge E.A., Wibrand F.
      Am. J. Hum. Genet. 81:383-387(2007) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN MTDPS9.
    6. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
      Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
      Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-54, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    8. "A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria."
      Ostergaard E., Schwartz M., Batbayli M., Christensen E., Hjalmarson O., Kollberg G., Holme E.
      Eur. J. Pediatr. 169:201-205(2010) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT MTDPS9 ALA-85.
    9. Cited for: VARIANT MTDPS9 ARG-170.
    10. Cited for: VARIANT MTDPS9 LEU-14.

    Entry informationi

    Entry nameiSUCA_HUMAN
    AccessioniPrimary (citable) accession number: P53597
    Secondary accession number(s): Q9BWB0, Q9UNP6
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1996
    Last sequence update: February 10, 2009
    Last modified: October 1, 2014
    This is version 149 of the entry and version 4 of the sequence. [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

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 2
      Human chromosome 2: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3