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Protein

ATP synthase F(0) complex subunit C1, mitochondrial

Gene

ATP5MC1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein 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
Sitei119Reversibly 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-1268020 Mitochondrial protein import
R-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 C1, mitochondrialCurated
Alternative name(s):
ATP synthase lipid-binding protein
ATP synthase membrane subunit c locus 1Imported
ATP synthase proteolipid P1
ATP synthase proton-transporting mitochondrial F(0) complex subunit C1
ATPase protein 9
ATPase subunit c
Gene namesi
Name:ATP5MC1Imported
Synonyms:ATP5G1Imported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

EuPathDBiHostDB:ENSG00000159199.13
HGNCiHGNC:841 ATP5MC1
MIMi603192 gene
neXtProtiNX_P05496

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
Transmembranei77 – 97HelicalSequence analysisAdd BLAST21
Transmembranei112 – 132HelicalSequence analysisAdd BLAST21

Keywords - Cellular componenti

CF(0), Membrane, Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi516
OpenTargetsiENSG00000159199
PharmGKBiPA25131

Polymorphism and mutation databases

BioMutaiATP5G1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 61MitochondrionAdd BLAST61
ChainiPRO_000000255762 – 136ATP synthase F(0) complex subunit C1, mitochondrialAdd BLAST75

Proteomic databases

PaxDbiP05496
PeptideAtlasiP05496
PRIDEiP05496
TopDownProteomicsiP05496

PTM databases

iPTMnetiP05496
PhosphoSitePlusiP05496

Expressioni

Gene expression databases

BgeeiENSG00000159199
CleanExiHS_ATP5G1
ExpressionAtlasiP05496 baseline and differential
GenevisibleiP05496 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. Component of an ATP synthase complex composed of ATP5F1, ATP5MC1, ATP5F1E, ATP5H, ATP5I, ATP5J, ATP5J2, MT-ATP6, MT-ATP8, ATP5F1A, ATP5F1B, ATP5F1D, ATP5F1C, ATP5O, ATP5L, USMG5 and MP68 (By similarity).By similarity

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi107001, 10 interactors
CORUMiP05496
IntActiP05496, 2 interactors
STRINGi9606.ENSP00000348205

Structurei

3D structure databases

ProteinModelPortaliP05496
SMRiP05496
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
InParanoidiP05496
KOiK02128
OMAiIRCCTRD
OrthoDBiEOG091G13J1
PhylomeDBiP05496
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.

P05496-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MQTAGALFIS PALIRCCTRG LIRPVSASFL NSPVNSSKQP SYSNFPLQVA
60 70 80 90 100
RREFQTSVVS RDIDTAAKFI GAGAATVGVA GSGAGIGTVF GSLIIGYARN
110 120 130
PSLKQQLFSY AILGFALSEA MGLFCLMVAF LILFAM
Length:136
Mass (Da):14,277
Last modified:February 1, 1994 - v2
Checksum:i1AFF1F16BB532647
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti53E → G in AAA51806 (PubMed:2883974).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X69907 Genomic DNA Translation: CAA49532.1
D13118 mRNA Translation: BAA02420.1
AL080089 mRNA Translation: CAB45704.1
BT007230 mRNA Translation: AAP35894.1
BC004963 mRNA Translation: AAH04963.1
M16453 mRNA Translation: AAA51806.1
CCDSiCCDS11539.1
PIRiS34066
RefSeqiNP_001002027.1, NM_001002027.1
NP_005166.1, NM_005175.2
UniGeneiHs.80986

Genome annotation databases

EnsembliENST00000355938; ENSP00000348205; ENSG00000159199
ENST00000393366; ENSP00000377033; ENSG00000159199
GeneIDi516
KEGGihsa:516

Similar proteinsi

Entry informationi

Entry nameiAT5G1_HUMAN
AccessioniPrimary (citable) accession number: P05496
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1988
Last sequence update: February 1, 1994
Last modified: May 23, 2018
This is version 175 of the entry and version 2 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

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