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Identification
HMDB Protein ID HMDBP01282
Secondary Accession Numbers
  • 6578
Name ATP synthase-coupling factor 6, mitochondrial
Synonyms
  1. ATPase subunit F6
Gene Name ATP5J
Protein Type Enzyme
Biological Properties
General Function Involved in hydrogen ion transmembrane transporter activity
Specific Function Mitochondrial membrane ATP synthase (F(1)F(0) 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, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Part of the complex F(0) domain and the peripheric stalk, which acts as a stator to hold the catalytic alpha(3)beta(3) subcomplex and subunit a/ATP6 static relative to the rotary elements. Also involved in the restoration of oligomycin-sensitive ATPase activity to depleted F1-F0 complexes
Pathways Not Available
Reactions Not Available
GO Classification
Component
cell part
membrane part
mitochondrial proton-transporting atp synthase complex, coupling factor f(o)
mitochondrial membrane part
Function
transmembrane transporter activity
substrate-specific transmembrane transporter activity
ion transmembrane transporter activity
cation transmembrane transporter activity
inorganic cation transmembrane transporter activity
monovalent inorganic cation transmembrane transporter activity
hydrogen ion transmembrane transporter activity
transporter activity
Process
purine nucleotide metabolic process
purine nucleotide biosynthetic process
purine nucleoside triphosphate biosynthetic process
purine ribonucleoside triphosphate biosynthetic process
metabolic process
nitrogen compound metabolic process
cellular nitrogen compound metabolic process
nucleobase, nucleoside, nucleotide and nucleic acid metabolic process
nucleobase, nucleoside and nucleotide metabolic process
nucleoside phosphate metabolic process
nucleotide metabolic process
atp synthesis coupled proton transport
atp biosynthetic process
Cellular Location
  1. Mitochondrion
  2. Mitochondrion inner membrane
Gene Properties
Chromosome Location Chromosome:2
Locus 21q21.1
SNPs ATP5J
Gene Sequence
>327 bp
ATGATTCTTCAGAGGCTCTTCAGGTTCTCCTCTGTCATTCGGTCAGCCGTCTCAGTCCAT
TTGCGGAGGAACATTGGTGTTACAGCAGTGGCATTTAATAAGGAACTTGATCCTATACAG
AAACTCTTTGTGGACAAGATTAGAGAATACAAATCTAAGCGACAGACATCTGGAGGACCT
GTTGATGCTAGTTCAGAGTATCAGCAAGAGCTGGAGAGGGAGCTTTTTAAGCTCAAGCAA
ATGTTTGGTAATGCAGACATGAATACATTTCCCACCTTCAAATTTGAAGATCCCAAATTT
GAAGTCATCGAAAAACCCCAGGCCTGA
Protein Properties
Number of Residues 108
Molecular Weight 12587.4
Theoretical pI 10.17
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>ATP synthase-coupling factor 6, mitochondrial
MILQRLFRFSSVIRSAVSVHLRRNIGVTAVAFNKELDPIQKLFVDKIREYKSKRQTSGGP
VDASSEYQQELERELFKLKQMFGNADMNTFPTFKFEDPKFEVIEKPQA
GenBank ID Protein 5817096
UniProtKB/Swiss-Prot ID P18859
UniProtKB/Swiss-Prot Entry Name ATP5J_HUMAN
PDB IDs Not Available
GenBank Gene ID AL110183
GeneCard ID ATP5J
GenAtlas ID ATP5J
HGNC ID HGNC:847
References
General References
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  2. Hochstrasser DF, Frutiger S, Paquet N, Bairoch A, Ravier F, Pasquali C, Sanchez JC, Tissot JD, Bjellqvist B, Vargas R, et al.: Human liver protein map: a reference database established by microsequencing and gel comparison. Electrophoresis. 1992 Dec;13(12):992-1001. [PubMed:1286669 ]
  3. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  4. Wiemann S, Weil B, Wellenreuther R, Gassenhuber J, Glassl S, Ansorge W, Bocher M, Blocker H, Bauersachs S, Blum H, Lauber J, Dusterhoft A, Beyer A, Kohrer K, Strack N, Mewes HW, Ottenwalder B, Obermaier B, Tampe J, Heubner D, Wambutt R, Korn B, Klein M, Poustka A: Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs. Genome Res. 2001 Mar;11(3):422-35. [PubMed:11230166 ]
  5. Hattori M, Fujiyama A, Taylor TD, Watanabe H, Yada T, Park HS, Toyoda A, Ishii K, Totoki Y, Choi DK, Groner Y, Soeda E, Ohki M, Takagi T, Sakaki Y, Taudien S, Blechschmidt K, Polley A, Menzel U, Delabar J, Kumpf K, Lehmann R, Patterson D, Reichwald K, Rump A, Schillhabel M, Schudy A, Zimmermann W, Rosenthal A, Kudoh J, Schibuya K, Kawasaki K, Asakawa S, Shintani A, Sasaki T, Nagamine K, Mitsuyama S, Antonarakis SE, Minoshima S, Shimizu N, Nordsiek G, Hornischer K, Brant P, Scharfe M, Schon O, Desario A, Reichelt J, Kauer G, Blocker H, Ramser J, Beck A, Klages S, Hennig S, Riesselmann L, Dagand E, Haaf T, Wehrmeyer S, Borzym K, Gardiner K, Nizetic D, Francis F, Lehrach H, Reinhardt R, Yaspo ML: The DNA sequence of human chromosome 21. Nature. 2000 May 18;405(6784):311-9. [PubMed:10830953 ]
  6. Javed AA, Ogata K, Sanadi DR: Human mitochondrial ATP synthase: cloning cDNA for the nuclear-encoded precursor of coupling factor 6. Gene. 1991 Jan 15;97(2):307-10. [PubMed:1825642 ]
  7. Higuti T, Tsurumi C, Kawamura Y, Tsujita H, Osaka F, Yoshihara Y, Tani I, Tanaka K, Ichihara A: Molecular cloning of cDNA for the import precursor of human coupling factor 6 of H(+)-ATP synthase in mitochondria. Biochem Biophys Res Commun. 1991 Jul 31;178(2):793-9. [PubMed:1830479 ]