ATP5MC3

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 key	Position(s)	DescriptionActions	Graphical view	Length
Siteⁱ	125	Reversibly protonated during proton transportBy similarity		1

GO - Molecular functionⁱ

lipid binding Source: UniProtKB-KW
proton-transporting ATP synthase activity, rotational mechanism
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
transporter activity
Source: ProtInc
Non-traceable author statementⁱ
- 7698763

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

ATP biosynthetic process Source: Reactome
ATP hydrolysis coupled proton transport Source: InterPro
ATP synthesis coupled proton transport
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
cristae formation Source: Reactome

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Biological process	Hydrogen ion transport, Ion transport, Transport
Ligand	Lipid-binding

Enzyme and pathway databases

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

Reactomeⁱ

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 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
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 2

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000154518.9
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:843 ATP5MC3
MIMⁱ	602736 gene
neXtProtⁱ	NX_P48201

Topology

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transmembraneⁱ	83 – 103	HelicalSequence analysisAdd BLAST		21
Transmembraneⁱ	118 – 138	HelicalSequence analysisAdd BLAST		21

Keywords - Cellular componentⁱ

CF(0), Membrane, Mitochondrion

Pathology & Biotechⁱ

Organism-specific databases

DisGeNET More...DisGeNETⁱ	518
Open Targets More...OpenTargetsⁱ	ENSG00000154518
PharmGKBⁱ	PA25133

Polymorphism and mutation databases

BioMutaⁱ	ATP5G3
DMDMⁱ	1352048

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 67	MitochondrionAdd BLAST		67
ChainⁱPRO_0000002567	68 – 142	ATP synthase F(0) complex subunit C3, mitochondrialAdd BLAST		75

Proteomic databases

EPDⁱ	P48201
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P48201
PaxDbⁱ	P48201
PeptideAtlas More...PeptideAtlasⁱ	P48201
PRIDEⁱ	P48201
ProteomicsDBⁱ	55872
TopDownProteomicsⁱ	P48201

PTM databases

iPTMnetⁱ	P48201
PhosphoSitePlusⁱ	P48201

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000154518 Expressed in 242 organ(s), highest expression level in heart
CleanExⁱ	HS_ATP5G3
Genevisibleⁱ	P48201 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

BioGridⁱ	107003, 12 interactors
IntActⁱ	P48201, 1 interactor
STRINGⁱ	9606.ENSP00000284727

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	P48201
SMRⁱ	P48201
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the ATPase C chain family.Curated

Keywords - Domainⁱ

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGⁱ	KOG3025 Eukaryota COG0636 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000006210
HOGENOMⁱ	HOG000235246
HOVERGENⁱ	HBG050605
InParanoidⁱ	P48201
KEGG Orthology (KO) More...KOⁱ	K02128
OMAⁱ	CKMFACA
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G13J1
PhylomeDBⁱ	P48201
TreeFamⁱ	TF300140

Family and domain databases

Gene3Dⁱ	1.20.20.10, 1 hit
HAMAPⁱ	MF_01396 ATP_synth_c_bact, 1 hit
InterProⁱ	View 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
PANTHERⁱ	PTHR10031 PTHR10031, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00137 ATP-synt_C, 1 hit
PRINTSⁱ	PR00124 ATPASEC
SUPFAMⁱ	SSF81333 SSF81333, 1 hit
PROSITEⁱ	View 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]FASTA Add 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 key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_011922	93	G → 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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS2263.1
PIRⁱ	I38612
NCBI Reference Sequences More...RefSeqⁱ	NP_001002258.1, NM_001002258.4 NP_001680.1, NM_001689.4
UniGeneⁱ	Hs.429

Genome annotation databases

Ensemblⁱ	ENST00000284727; ENSP00000284727; ENSG00000154518 ENST00000392541; ENSP00000376324; ENSG00000154518 ENST00000409194; ENSP00000387317; ENSG00000154518
GeneIDⁱ	518
KEGGⁱ	hsa:518
UCSC genome browser More...UCSCⁱ	uc002ujz.5 human

Keywords - Coding sequence diversityⁱ

Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P48201	ATP synthase F(0) complex subunit C3, mitochondrial		RAT		142	UniRef100_P48201
ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C3 (Subunit 9)		PANTR		142
ATP synthase membrane subunit c locus 3		GORGO		142
Uncharacterized protein		PANPA		142
Uncharacterized protein		NOMLE		142
+1

Protein	Similar proteins		Species	Score	Length	Source
P48201	ATP synthase F(0) complex subunit C3, mitochondrial		RAT		142	UniRef90_P48201
ATP synthase F(0) complex subunit C3, mitochondrial		PONAB		142
ATP synthase F(0) complex subunit C3, mitochondrial		BOVIN		141
ATP synthase F(0) complex subunit C3, mitochondrial		MOUSE		141
ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C3 (Subunit 9)		PANTR		142
+115

Protein	Similar proteins		Species	Score	Length	Source
P48201	ATP synthase F(0) complex subunit C1, mitochondrial		HUMAN		136	UniRef50_P05496
ATP synthase F(0) complex subunit C3, mitochondrial		RAT		142
ATP synthase F(0) complex subunit C3, mitochondrial		PONAB		142
ATP synthase F(0) complex subunit C3, mitochondrial		MOUSE		141
ATP synthase F(0) complex subunit C3, mitochondrial		BOVIN		141
+259

Cross-referencesⁱ

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
CCDSⁱ	CCDS2263.1
PIRⁱ	I38612
RefSeqⁱ	NP_001002258.1, NM_001002258.4 NP_001680.1, NM_001689.4
UniGeneⁱ	Hs.429

3D structure databases

ProteinModelPortalⁱ	P48201
SMRⁱ	P48201
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	107003, 12 interactors
IntActⁱ	P48201, 1 interactor
STRINGⁱ	9606.ENSP00000284727

PTM databases

iPTMnetⁱ	P48201
PhosphoSitePlusⁱ	P48201

Polymorphism and mutation databases

BioMutaⁱ	ATP5G3
DMDMⁱ	1352048

Proteomic databases

EPDⁱ	P48201
MaxQBⁱ	P48201
PaxDbⁱ	P48201
PeptideAtlasⁱ	P48201
PRIDEⁱ	P48201
ProteomicsDBⁱ	55872
TopDownProteomicsⁱ	P48201

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	518
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000284727; ENSP00000284727; ENSG00000154518 ENST00000392541; ENSP00000376324; ENSG00000154518 ENST00000409194; ENSP00000387317; ENSG00000154518
GeneIDⁱ	518
KEGGⁱ	hsa:518
UCSCⁱ	uc002ujz.5 human

Organism-specific databases

CTDⁱ	518
DisGeNETⁱ	518
EuPathDBⁱ	HostDB:ENSG00000154518.9
GeneCardsⁱ	ATP5MC3
HGNCⁱ	HGNC:843 ATP5MC3
MIMⁱ	602736 gene
neXtProtⁱ	NX_P48201
OpenTargetsⁱ	ENSG00000154518
PharmGKBⁱ	PA25133
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG3025 Eukaryota COG0636 LUCA
GeneTreeⁱ	ENSGT00390000006210
HOGENOMⁱ	HOG000235246
HOVERGENⁱ	HBG050605
InParanoidⁱ	P48201
KOⁱ	K02128
OMAⁱ	CKMFACA
OrthoDBⁱ	EOG091G13J1
PhylomeDBⁱ	P48201
TreeFamⁱ	TF300140

Enzyme and pathway databases

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

Reactomeⁱ

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

Miscellaneous databases

ChiTaRSⁱ	ATP5G3 human
GeneWikiⁱ	ATP5G3
GenomeRNAiⁱ	518
Protein Ontology More...PROⁱ	PR:P48201
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000154518 Expressed in 242 organ(s), highest expression level in heart
CleanExⁱ	HS_ATP5G3
Genevisibleⁱ	P48201 HS

Family and domain databases

Gene3Dⁱ	1.20.20.10, 1 hit
HAMAPⁱ	MF_01396 ATP_synth_c_bact, 1 hit
InterProⁱ	View 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
PANTHERⁱ	PTHR10031 PTHR10031, 1 hit
Pfamⁱ	View protein in Pfam PF00137 ATP-synt_C, 1 hit
PRINTSⁱ	PR00124 ATPASEC
SUPFAMⁱ	SSF81333 SSF81333, 1 hit
PROSITEⁱ	View protein in PROSITE PS00605 ATPASE_C, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	AT5G3_HUMAN
Accessionⁱ	P48201Primary (citable) accession number: P48201 Secondary accession number(s): B2R4Z0, D3DPF0, Q4ZFX7
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	February 1, 1996
	Last sequence update:	February 1, 1996
	Last modified:	November 7, 2018
	This is version 160 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any 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

SIMILARITY comments
Index of protein domains and families
Human chromosome 2
Human chromosome 2: entries, gene names and cross-references to MIM
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

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UniParc

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UniRef

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Supporting data

UniProtKB - P48201 (AT5G3_HUMAN)

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ATP synthase F(0) complex subunit C3, mitochondrial

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Miscellaneous

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Mitochondrion

Mitochondrion

Other locations

Topology

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Natural variant

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ