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Protein

ATP synthase F(0) complex subunit C3, mitochondrial

Gene

ATP5MC3

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
Sitei125Reversibly protonated during proton transportBy similarity1

GO - Molecular functioni

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 C3, mitochondrialCurated
Alternative name(s):
ATP synthase lipid-binding protein
ATP synthase membrane subunit c locus 3Imported
ATP synthase proteolipid P3
ATP synthase proton-transporting mitochondrial F(0) complex subunit C3
ATPase protein 9
ATPase subunit c
Gene namesi
Name:ATP5MC3Imported
Synonyms:ATP5G3Imported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

EuPathDBiHostDB:ENSG00000154518.9
HGNCiHGNC:843 ATP5MC3
MIMi602736 gene
neXtProtiNX_P48201

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
Transmembranei83 – 103HelicalSequence analysisAdd BLAST21
Transmembranei118 – 138HelicalSequence analysisAdd BLAST21

Keywords - Cellular componenti

CF(0), Membrane, Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi518
OpenTargetsiENSG00000154518
PharmGKBiPA25133

Polymorphism and mutation databases

BioMutaiATP5G3
DMDMi1352048

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 67MitochondrionAdd BLAST67
ChainiPRO_000000256768 – 142ATP synthase F(0) complex subunit C3, mitochondrialAdd BLAST75

Proteomic databases

EPDiP48201
MaxQBiP48201
PaxDbiP48201
PeptideAtlasiP48201
PRIDEiP48201
ProteomicsDBi55872
TopDownProteomicsiP48201

PTM databases

iPTMnetiP48201
PhosphoSitePlusiP48201

Expressioni

Gene expression databases

BgeeiENSG00000154518
CleanExiHS_ATP5G3
GenevisibleiP48201 HS

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

BioGridi107003, 12 interactors
IntActiP48201, 1 interactor
STRINGi9606.ENSP00000284727

Structurei

3D structure databases

ProteinModelPortaliP48201
SMRiP48201
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
InParanoidiP48201
KOiK02128
OMAiCKMFACA
OrthoDBiEOG091G13J1
PhylomeDBiP48201
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

Sequencei

Sequence statusi: Complete.

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

P48201-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MFACAKLACT PSLIRAGSRV AYRPISASVL SRPEASRTGE GSTVFNGAQN
60 70 80 90 100
GVSQLIQREF QTSAISRDID TAAKFIGAGA ATVGVAGSGA GIGTVFGSLI
110 120 130 140
IGYARNPSLK QQLFSYAILG FALSEAMGLF CLMVAFLILF AM
Length:142
Mass (Da):14,693
Last modified:February 1, 1996 - v1
Checksum:i19EC0D1710A0AA3F
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01192293G → E. Corresponds to variant dbSNP:rs1802622Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U09813 mRNA Translation: AAA78807.1
AK311999 mRNA Translation: BAG34937.1
AC096649 Genomic DNA Translation: AAX88970.1
CH471058 Genomic DNA Translation: EAX11106.1
CH471058 Genomic DNA Translation: EAX11107.1
BC106881 mRNA Translation: AAI06882.1
CCDSiCCDS2263.1
PIRiI38612
RefSeqiNP_001002258.1, NM_001002258.4
NP_001680.1, NM_001689.4
UniGeneiHs.429

Genome annotation databases

EnsembliENST00000284727; ENSP00000284727; ENSG00000154518
ENST00000392541; ENSP00000376324; ENSG00000154518
ENST00000409194; ENSP00000387317; ENSG00000154518
GeneIDi518
KEGGihsa:518
UCSCiuc002ujz.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiAT5G3_HUMAN
AccessioniPrimary (citable) accession number: P48201
Secondary accession number(s): B2R4Z0, D3DPF0, Q4ZFX7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: February 1, 1996
Last modified: June 20, 2018
This is version 158 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. 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. SIMILARITY comments
    Index of protein domains and families

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