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Protein

ATP synthase F(0) complex subunit C2, mitochondrial

Gene

ATP5G2

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

Functioni

Mitochondrial membrane ATP synthase (F1F0 ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F1 - containing the extramembraneous catalytic core and F0 - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F1 is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Part of the complex F0 domain. A homomeric c-ring of probably 10 subunits is part of the complex rotary element.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei124Reversibly protonated during proton transportBy similarity1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Hydrogen ion transport, Ion transport, Transport

Keywords - Ligandi

Lipid-binding

Enzyme and pathway databases

BioCyciZFISH:HS05994-MONOMER.
ReactomeiR-HSA-163210. Formation of ATP by chemiosmotic coupling.

Names & Taxonomyi

Protein namesi
Recommended name:
ATP synthase F(0) complex subunit C2, mitochondrial
Alternative name(s):
ATP synthase lipid-binding protein
ATP synthase proteolipid P2
ATP synthase proton-transporting mitochondrial F(0) complex subunit C2
ATPase protein 9
ATPase subunit c
Gene namesi
Name:ATP5G2
ORF Names:PSEC0033
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:842. ATP5G2.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei82 – 102HelicalSequence analysisAdd BLAST21
Transmembranei117 – 137HelicalSequence analysisAdd BLAST21

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

CF(0), Membrane, Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi517.
OpenTargetsiENSG00000135390.
PharmGKBiPA25132.

Polymorphism and mutation databases

DMDMi461592.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 66MitochondrionBy similarityAdd BLAST66
ChainiPRO_000000256267 – 141ATP synthase F(0) complex subunit C2, mitochondrialAdd BLAST75

Proteomic databases

MaxQBiQ06055.
PaxDbiQ06055.
PRIDEiQ06055.
TopDownProteomicsiQ06055-1. [Q06055-1]
Q06055-2. [Q06055-2]

PTM databases

PhosphoSitePlusiQ06055.

Expressioni

Gene expression databases

BgeeiENSG00000135390.
CleanExiHS_ATP5G2.
ExpressionAtlasiQ06055. baseline and differential.
GenevisibleiQ06055. HS.

Organism-specific databases

HPAiHPA051469.

Interactioni

Subunit structurei

F-type ATPases have 2 components, CF1 - the catalytic core - and CF0 - the membrane proton channel. CF1 has five subunits: alpha3, beta3, gamma1, delta1, epsilon1. CF0 has three main subunits: a, b and c.

Protein-protein interaction databases

BioGridi107002. 3 interactors.
IntActiQ06055. 3 interactors.
STRINGi9606.ENSP00000377878.

Structurei

3D structure databases

ProteinModelPortaliQ06055.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the ATPase C chain family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3025. Eukaryota.
COG0636. LUCA.
GeneTreeiENSGT00390000006210.
HOGENOMiHOG000235246.
HOVERGENiHBG050605.
InParanoidiQ06055.
KOiK02128.
OMAiHPLKMYT.
OrthoDBiEOG091G13J1.
PhylomeDBiQ06055.
TreeFamiTF300140.

Family and domain databases

Gene3Di1.20.20.10. 1 hit.
HAMAPiMF_01396. ATP_synth_c_bact. 1 hit.
InterProiIPR000454. ATP_synth_F0_csu.
IPR020537. ATP_synth_F0_csu_DDCD_BS.
IPR002379. ATPase_proteolipid_c-like_dom.
[Graphical view]
PfamiPF00137. ATP-synt_C. 1 hit.
[Graphical view]
PRINTSiPR00124. ATPASEC.
SUPFAMiSSF81333. SSF81333. 1 hit.
PROSITEiPS00605. ATPASE_C. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q06055-1) [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
MFACSKFVST PSLVKSTSQL LSRPLSAVVL KRPEILTDES LSSLAVSCPL
60 70 80 90 100
TSLVSSRSFQ TSAISRDIDT AAKFIGAGAA TVGVAGSGAG IGTVFGSLII
110 120 130 140
GYARNPSLKQ QLFSYAILGF ALSEAMGLFC LMVAFLILFA M
Length:141
Mass (Da):14,637
Last modified:February 1, 1994 - v1
Checksum:i6E627A504A7AE52D
GO
Isoform 2 (identifier: Q06055-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MPELILYVAITLSVAERLVGPGHACAEPSFRSSRCSAPLCLLCSGSSSPATAPHPLKM

Note: Derived from EST data.
Show »
Length:198
Mass (Da):20,513
Checksum:i6ACCBD405F313CC8
GO
Isoform 3 (identifier: Q06055-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MPELILSPATAPHPLKM

Note: Derived from EST data.
Show »
Length:157
Mass (Da):16,334
Checksum:i84032DCC16E10CED
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti63A → T in BAG52118 (PubMed:16303743).Curated1
Sequence conflicti107S → F in BAG52118 (PubMed:16303743).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01192058S → I.Corresponds to variant rs13819dbSNPEnsembl.1
Natural variantiVAR_011921141M → K.Corresponds to variant rs1803177dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0373481M → MPELILYVAITLSVAERLVG PGHACAEPSFRSSRCSAPLC LLCSGSSSPATAPHPLKM in isoform 2. Curated1
Alternative sequenceiVSP_0373491M → MPELILSPATAPHPLKM in isoform 3. Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X69908 Genomic DNA. Translation: CAA49533.1.
D13119 mRNA. Translation: BAA02421.1.
AK075351 mRNA. Translation: BAG52118.1.
AC073594 Genomic DNA. No translation available.
BC020826 mRNA. Translation: AAH20826.1.
CCDSiCCDS31812.1. [Q06055-3]
CCDS81694.1. [Q06055-1]
CCDS8863.2. [Q06055-2]
PIRiS34067.
RefSeqiNP_001002031.1. NM_001002031.2. [Q06055-3]
NP_001317198.1. NM_001330269.1.
NP_005167.2. NM_005176.5. [Q06055-2]
XP_016874949.1. XM_017019460.1. [Q06055-2]
XP_016874950.1. XM_017019461.1. [Q06055-3]
UniGeneiHs.524464.

Genome annotation databases

EnsembliENST00000338662; ENSP00000340315; ENSG00000135390. [Q06055-3]
ENST00000394349; ENSP00000377878; ENSG00000135390. [Q06055-2]
ENST00000549164; ENSP00000447317; ENSG00000135390. [Q06055-1]
ENST00000552242; ENSP00000448801; ENSG00000135390. [Q06055-1]
ENST00000602871; ENSP00000473535; ENSG00000135390. [Q06055-1]
GeneIDi517.
KEGGihsa:517.
UCSCiuc001sec.4. human. [Q06055-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X69908 Genomic DNA. Translation: CAA49533.1.
D13119 mRNA. Translation: BAA02421.1.
AK075351 mRNA. Translation: BAG52118.1.
AC073594 Genomic DNA. No translation available.
BC020826 mRNA. Translation: AAH20826.1.
CCDSiCCDS31812.1. [Q06055-3]
CCDS81694.1. [Q06055-1]
CCDS8863.2. [Q06055-2]
PIRiS34067.
RefSeqiNP_001002031.1. NM_001002031.2. [Q06055-3]
NP_001317198.1. NM_001330269.1.
NP_005167.2. NM_005176.5. [Q06055-2]
XP_016874949.1. XM_017019460.1. [Q06055-2]
XP_016874950.1. XM_017019461.1. [Q06055-3]
UniGeneiHs.524464.

3D structure databases

ProteinModelPortaliQ06055.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107002. 3 interactors.
IntActiQ06055. 3 interactors.
STRINGi9606.ENSP00000377878.

PTM databases

PhosphoSitePlusiQ06055.

Polymorphism and mutation databases

DMDMi461592.

Proteomic databases

MaxQBiQ06055.
PaxDbiQ06055.
PRIDEiQ06055.
TopDownProteomicsiQ06055-1. [Q06055-1]
Q06055-2. [Q06055-2]

Protocols and materials databases

DNASUi517.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000338662; ENSP00000340315; ENSG00000135390. [Q06055-3]
ENST00000394349; ENSP00000377878; ENSG00000135390. [Q06055-2]
ENST00000549164; ENSP00000447317; ENSG00000135390. [Q06055-1]
ENST00000552242; ENSP00000448801; ENSG00000135390. [Q06055-1]
ENST00000602871; ENSP00000473535; ENSG00000135390. [Q06055-1]
GeneIDi517.
KEGGihsa:517.
UCSCiuc001sec.4. human. [Q06055-1]

Organism-specific databases

CTDi517.
DisGeNETi517.
GeneCardsiATP5G2.
H-InvDBHIX0201895.
HGNCiHGNC:842. ATP5G2.
HPAiHPA051469.
MIMi603193. gene.
neXtProtiNX_Q06055.
OpenTargetsiENSG00000135390.
PharmGKBiPA25132.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3025. Eukaryota.
COG0636. LUCA.
GeneTreeiENSGT00390000006210.
HOGENOMiHOG000235246.
HOVERGENiHBG050605.
InParanoidiQ06055.
KOiK02128.
OMAiHPLKMYT.
OrthoDBiEOG091G13J1.
PhylomeDBiQ06055.
TreeFamiTF300140.

Enzyme and pathway databases

BioCyciZFISH:HS05994-MONOMER.
ReactomeiR-HSA-163210. Formation of ATP by chemiosmotic coupling.

Miscellaneous databases

ChiTaRSiATP5G2. human.
GeneWikiiATP5G2.
GenomeRNAii517.
PROiQ06055.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000135390.
CleanExiHS_ATP5G2.
ExpressionAtlasiQ06055. baseline and differential.
GenevisibleiQ06055. HS.

Family and domain databases

Gene3Di1.20.20.10. 1 hit.
HAMAPiMF_01396. ATP_synth_c_bact. 1 hit.
InterProiIPR000454. ATP_synth_F0_csu.
IPR020537. ATP_synth_F0_csu_DDCD_BS.
IPR002379. ATPase_proteolipid_c-like_dom.
[Graphical view]
PfamiPF00137. ATP-synt_C. 1 hit.
[Graphical view]
PRINTSiPR00124. ATPASEC.
SUPFAMiSSF81333. SSF81333. 1 hit.
PROSITEiPS00605. ATPASE_C. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiAT5G2_HUMAN
AccessioniPrimary (citable) accession number: Q06055
Secondary accession number(s): B3KQQ6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1994
Last sequence update: February 1, 1994
Last modified: November 30, 2016
This is version 153 of the entry and version 1 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

Miscellaneous

There are three genes which encode the mitochondrial ATP synthase proteolipid and they specify precursors with different import sequences but identical mature proteins. Is the major protein stored in the storage bodies of animals or humans affected with ceroid lipofuscinosis (Batten disease).

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 12
    Human chromosome 12: 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. SIMILARITY comments
    Index of protein domains and families

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 one 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.