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Protein

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

Gene

SUCLG1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

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

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Biological processi

Tricarboxylic acid cycle

Keywords - Ligandi

GTP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS08877-MONOMER.
BRENDAi6.2.1.4. 2681.
6.2.1.5. 2681.
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 Componenti: Chromosome 2

Organism-specific databases

HGNCiHGNC:11449. SUCLG1.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Mitochondrial DNA depletion syndrome 9 (MTDPS9)4 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionA 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.

See also OMIM:245400
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.

Chemistry

DrugBankiDB00139. Succinic acid.

Polymorphism and mutation databases

BioMutaiSUCLG1.
DMDMi223634731.

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

BgeeiP53597.
CleanExiHS_SUCLG1.
ExpressionAtlasiP53597. baseline and differential.
GenevestigatoriP53597.

Organism-specific databases

HPAiHPA036683.
HPA036684.

Interactioni

Subunit structurei

Heterodimer of an alpha and a beta subunit.

Protein-protein interaction databases

BioGridi114330. 17 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.
GeneTreeiENSGT00530000063275.
HOGENOMiHOG000239685.
HOVERGENiHBG000957.
InParanoidiP53597.
KOiK01899.
OMAiITEHIPI.
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

        10         20         30         40         50
MTATLAAAAD IATMVSGSSG LAAARLLSRS FLLPQNGIRH CSYTASRQHL
60 70 80 90 100
YVDKNTKIIC QGFTGKQGTF HSQQALEYGT KLVGGTTPGK GGQTHLGLPV
110 120 130 140 150
FNTVKEAKEQ TGATASVIYV PPPFAAAAIN EAIEAEIPLV VCITEGIPQQ
160 170 180 190 200
DMVRVKHKLL RQEKTRLIGP NCPGVINPGE CKIGIMPGHI HKKGRIGIVS
210 220 230 240 250
RSGTLTYEAV HQTTQVGLGQ SLCVGIGGDP FNGTDFIDCL EIFLNDSATE
260 270 280 290 300
GIILIGEIGG NAEENAAEFL KQHNSGPNSK PVVSFIAGLT APPGRRMGHA
310 320 330 340
GAIIAGGKGG AKEKISALQS AGVVVSMSPA QLGTTIYKEF EKRKML
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. Curated
The sequence AAH00504.1 differs from that shown. Reason: Erroneous initiation. Curated
The sequence CAA92426.1 differs from that shown. Reason: Erroneous initiation. Curated

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 (Ref. 3) Curated

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:
EMBLi
GenBanki
DDBJi
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.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

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

Protein-protein interaction databases

BioGridi114330. 17 interactions.
IntActiP53597. 1 interaction.
MINTiMINT-137948.
STRINGi9606.ENSP00000377446.

Chemistry

DrugBankiDB00139. Succinic acid.

PTM databases

PhosphoSiteiP53597.

Polymorphism and mutation databases

BioMutaiSUCLG1.
DMDMi223634731.

2D gel databases

UCD-2DPAGEP53597.

Proteomic databases

MaxQBiP53597.
PaxDbiP53597.
PRIDEiP53597.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi8802.
GeneCardsiGC02M084562.
HGNCiHGNC:11449. SUCLG1.
HPAiHPA036683.
HPA036684.
MIMi245400. phenotype.
611224. gene.
neXtProtiNX_P53597.
Orphaneti17. Fatal infantile lactic acidosis with methylmalonic aciduria.
PharmGKBiPA36246.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG0074.
GeneTreeiENSGT00530000063275.
HOGENOMiHOG000239685.
HOVERGENiHBG000957.
InParanoidiP53597.
KOiK01899.
OMAiITEHIPI.
OrthoDBiEOG74BJSG.
PhylomeDBiP53597.
TreeFamiTF300666.

Enzyme and pathway databases

UniPathwayiUPA00223.
BioCyciMetaCyc:HS08877-MONOMER.
BRENDAi6.2.1.4. 2681.
6.2.1.5. 2681.
ReactomeiREACT_1785. Citric acid cycle (TCA cycle).

Miscellaneous databases

ChiTaRSiSUCLG1. human.
GeneWikiiSUCLG1.
GenomeRNAii8802.
NextBioi33016.
PROiP53597.
SOURCEiSearch...

Gene expression databases

BgeeiP53597.
CleanExiHS_SUCLG1.
ExpressionAtlasiP53597. baseline and differential.
GenevestigatoriP53597.

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]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  9. "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.
  10. Cited for: VARIANT MTDPS9 ARG-170.
  11. 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: May 27, 2015
This is version 156 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.