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Protein

ATP synthase F(0) complex subunit C2, mitochondrial

Gene

ATP5MC2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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.

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).

Sites

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

GO - Molecular functioni

  • lipid binding Source: UniProtKB-KW
  • proton-transporting ATP synthase activity, rotational mechanism Source: GO_Central
  • transporter activity Source: ProtInc

GO - Biological processi

Keywordsi

Biological processHydrogen ion transport, Ion transport, Transport
LigandLipid-binding

Enzyme and pathway databases

ReactomeiR-HSA-163210 Formation of ATP by chemiosmotic coupling
R-HSA-8949613 Cristae formation

Names & Taxonomyi

Protein namesi
Recommended name:
ATP synthase F(0) complex subunit C2, mitochondrialCurated
Alternative name(s):
ATP synthase lipid-binding protein
ATP synthase membrane subunit c locus 2Imported
ATP synthase proteolipid P2
ATP synthase proton-transporting mitochondrial F(0) complex subunit C2
ATPase protein 9
ATPase subunit c
Gene namesi
Name:ATP5MC2Imported
Synonyms:ATP5G2Imported
ORF Names:PSEC0033
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

EuPathDBiHostDB:ENSG00000135390.17
HGNCiHGNC:842 ATP5MC2
MIMi603193 gene
neXtProtiNX_Q06055

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

Topology

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

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

EPDiQ06055
MaxQBiQ06055
PaxDbiQ06055
PeptideAtlasiQ06055
PRIDEiQ06055
ProteomicsDBi58411
58412 [Q06055-2]
58413 [Q06055-3]
TopDownProteomicsiQ06055-1 [Q06055-1]
Q06055-2 [Q06055-2]
Q06055-3 [Q06055-3]

PTM databases

iPTMnetiQ06055
PhosphoSitePlusiQ06055

Expressioni

Gene expression databases

BgeeiENSG00000135390 Expressed in 232 organ(s), highest expression level in apex of heart
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
SMRiQ06055
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
OMAiMYTCSRF
OrthoDBiEOG091G13J1
PhylomeDBiQ06055
TreeFamiTF300140

Family and domain databases

Gene3Di1.20.20.10, 1 hit
HAMAPiMF_01396 ATP_synth_c_bact, 1 hit
InterProiView protein in InterPro
IPR000454 ATP_synth_F0_csu
IPR020537 ATP_synth_F0_csu_DDCD_BS
IPR038662 ATP_synth_F0_csu_sf
IPR002379 ATPase_proteolipid_c-like_dom
IPR035921 F/V-ATP_Csub_sf
PANTHERiPTHR10031 PTHR10031, 1 hit
PfamiView protein in Pfam
PF00137 ATP-synt_C, 1 hit
PRINTSiPR00124 ATPASEC
SUPFAMiSSF81333 SSF81333, 1 hit
PROSITEiView protein in PROSITE
PS00605 ATPASE_C, 1 hit

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

This entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F8W041F8W041_HUMAN
ATP synthase F(0) complex subunit C...
ATP5MC2
16Annotation score:

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 dbSNP:rs13819Ensembl.1
Natural variantiVAR_011921141M → K. Corresponds to variant dbSNP:rs1803177Ensembl.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]
PIRiS34067
RefSeqiNP_001002031.1, NM_001002031.2 [Q06055-3]
NP_001317198.1, NM_001330269.1 [Q06055-1]
NP_005167.2, NM_005176.5 [Q06055-1]
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

Similar proteinsi

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]
PIRiS34067
RefSeqiNP_001002031.1, NM_001002031.2 [Q06055-3]
NP_001317198.1, NM_001330269.1 [Q06055-1]
NP_005167.2, NM_005176.5 [Q06055-1]
XP_016874949.1, XM_017019460.1 [Q06055-2]
XP_016874950.1, XM_017019461.1 [Q06055-3]
UniGeneiHs.524464

3D structure databases

ProteinModelPortaliQ06055
SMRiQ06055
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107002, 3 interactors
IntActiQ06055, 3 interactors
STRINGi9606.ENSP00000377878

PTM databases

iPTMnetiQ06055
PhosphoSitePlusiQ06055

Polymorphism and mutation databases

DMDMi461592

Proteomic databases

EPDiQ06055
MaxQBiQ06055
PaxDbiQ06055
PeptideAtlasiQ06055
PRIDEiQ06055
ProteomicsDBi58411
58412 [Q06055-2]
58413 [Q06055-3]
TopDownProteomicsiQ06055-1 [Q06055-1]
Q06055-2 [Q06055-2]
Q06055-3 [Q06055-3]

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
EuPathDBiHostDB:ENSG00000135390.17
GeneCardsiATP5MC2
H-InvDBiHIX0201895
HGNCiHGNC:842 ATP5MC2
HPAiHPA051469
MIMi603193 gene
neXtProtiNX_Q06055
OpenTargetsiENSG00000135390
PharmGKBiPA25132
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-HSA-163210 Formation of ATP by chemiosmotic coupling
R-HSA-8949613 Cristae formation

Miscellaneous databases

ChiTaRSiATP5G2 human
GeneWikiiATP5G2
GenomeRNAii517
PROiPR:Q06055
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000135390 Expressed in 232 organ(s), highest expression level in apex of heart
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
InterProiView protein in InterPro
IPR000454 ATP_synth_F0_csu
IPR020537 ATP_synth_F0_csu_DDCD_BS
IPR038662 ATP_synth_F0_csu_sf
IPR002379 ATPase_proteolipid_c-like_dom
IPR035921 F/V-ATP_Csub_sf
PANTHERiPTHR10031 PTHR10031, 1 hit
PfamiView protein in Pfam
PF00137 ATP-synt_C, 1 hit
PRINTSiPR00124 ATPASEC
SUPFAMiSSF81333 SSF81333, 1 hit
PROSITEiView protein in PROSITE
PS00605 ATPASE_C, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiAT5G2_HUMAN
AccessioniPrimary (citable) accession number: Q06055
Secondary accession number(s): B3KQQ6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1994
Last sequence update: February 1, 1994
Last modified: November 7, 2018
This is version 169 of the entry and version 1 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

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
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