Q96MF6 (CQ10A_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Coenzyme Q-binding protein COQ10 homolog A, mitochondrial

Gene names

Name:	COQ10A
ORF Names:	UNQ6192/PRO20219

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	247 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at transcript level

General annotation (Comments)

Function	Required for the function of coenzyme Q in the respiratory chain. May serve as a chaperone or may be involved in the transport of Q6 from its site of synthesis to the catalytic sites of the respiratory complexes Probable. Ref.4
Subunit structure	Interacts with coenzyme Q By similarity.
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein; Matrix side By similarity.
Sequence similarities	Belongs to the COQ10 family.
Sequence caution	The sequence AAQ89220.1 differs from that shown. Reason: Erroneous initiation.

Ontologies

Keywords
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Transit peptide
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Cellular_component	mitochondrial inner membrane Inferred from electronic annotation. Source: UniProtKB-SubCell
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q96MF6-1)

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.

Isoform 2 (identifier: Q96MF6-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-44: MAWAGSRRVPAGTRAAAERCCRLSLSPGAQPAPPPGPLPPPRPM → MHSSSPSPTNDRGLRRTIRLAPLSFGL

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 15	15	Mitochondrion Potential
	Chain	16 – 247	232	Coenzyme Q-binding protein COQ10 homolog A, mitochondrial		PRO_0000228644
Natural variations
	Alternative sequence	1 – 44	44	MAWAG…PPRPM → MHSSSPSPTNDRGLRRTIRL APLSFGL in isoform 2.		VSP_017685
	Natural variant	79	1	P → H. Ref.2 Corresponds to variant rs11543258 [ dbSNP | Ensembl ].		VAR_025703
	Natural variant	231	1	P → S. Corresponds to variant rs3184994 [ dbSNP | Ensembl ].		VAR_048828
Experimental info
	Sequence conflict	38	1	L → P in BAB71339. Ref.1
	Sequence conflict	202	1	H → R in BAB71339. Ref.1
	Sequence conflict	213	1	V → A in BAB71339. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified March 21, 2006. Version 2. Checksum: 148299DE0704A8B8	FASTA	247	27,686
	10 20 30 40 50 60 MAWAGSRRVP AGTRAAAERC CRLSLSPGAQ PAPPPGPLPP PRPMRFLTSC SLLLPRAAQI 70 80 90 100 110 120 LAAEAGLPSS RSFMGFAAPF TNKRKAYSER RIMGYSMQEM YEVVSNVQEY REFVPWCKKS 130 140 150 160 170 180 LVVSSRKGHL KAQLEVGFPP VMERYTSAVS MVKPHMVKAV CTDGKLFNHL ETIWRFSPGI 190 200 210 220 230 240 PAYPRTCTVD FSISFEFRSL LHSQLATMFF DEVVKQNVAA FERRAATKFG PETAIPRELM FHEVHQT
	Isoform 2 [UniParc]. Checksum: AD69752664DF5F99	FASTA	230	26,120
	10 20 30 40 50 60 MHSSSPSPTN DRGLRRTIRL APLSFGLRFL TSCSLLLPRA AQILAAEAGL PSSRSFMGFA 70 80 90 100 110 120 APFTNKRKAY SERRIMGYSM QEMYEVVSNV QEYREFVPWC KKSLVVSSRK GHLKAQLEVG 130 140 150 160 170 180 FPPVMERYTS AVSMVKPHMV KAVCTDGKLF NHLETIWRFS PGIPAYPRTC TVDFSISFEF 190 200 210 220 230 RSLLHSQLAT MFFDEVVKQN VAAFERRAAT KFGPETAIPR ELMFHEVHQT

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2). Tissue: Skeletal muscle.
[2]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), VARIANT HIS-79. Tissue: Brain, Colon and Skin.
[3]	"The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment." Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J., Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P., Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E. , Heldens S., Huang A., Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D., Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L., Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C., Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J., Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M. Genome Res. 13:2265-2270(2003) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 18-247 (ISOFORM 1).
[4]	"The Saccharomyces cerevisiae COQ10 gene encodes a START domain protein required for function of coenzyme Q in respiration." Barros M.H., Johnson A., Gin P., Marbois B.N., Clarke C.F., Tzagoloff A. J. Biol. Chem. 280:42627-42635(2005) [PubMed] [Europe PMC] [Abstract] Cited for: PROBABLE FUNCTION.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AK057003 mRNA. Translation: BAB71339.1. AK057014 mRNA. Translation: BAB71344.1. BC002435 mRNA. Translation: AAH02435.2. BC047444 mRNA. Translation: AAH47444.1. BC073923 mRNA. Translation: AAH73923.1. AY358861 mRNA. Translation: AAQ89220.1. Different initiation.
IPI	IPI00065495. IPI00736624.
RefSeq	NP_653177.3. NM_144576.3.
UniGene	Hs.4096.
3D structure databases
ProteinModelPortal	Q96MF6.
ModBase	Search...
Protein-protein interaction databases
MINT	MINT-6942683.
STRING	9606.ENSP00000312587.
PTM databases
PhosphoSite	Q96MF6.
Polymorphism databases
DMDM	90111993.
Proteomic databases
PaxDb	Q96MF6.
PRIDE	Q96MF6.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000308197; ENSP00000312587; ENSG00000135469. ENST00000546544; ENSP00000446723; ENSG00000135469.
GeneID	93058.
KEGG	hsa:93058.
UCSC	uc001sko.4. human. uc001skq.4. human.
Organism-specific databases
CTD	93058.
GeneCards	GC12P056660.
HGNC	HGNC:26515. COQ10A.
neXtProt	NX_Q96MF6.
PharmGKB	PA143485439.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG2867.
HOGENOM	HOG000261732.
HOVERGEN	HBG081329.
OMA	MQEMFEV.
OrthoDB	EOG4Q84Z9.
Gene expression databases
ArrayExpress	Q96MF6.
Bgee	Q96MF6.
CleanEx	HS_COQ10A.
Genevestigator	Q96MF6.
GermOnline	ENSG00000135469. Homo sapiens.
Family and domain databases
Gene3D	3.30.530.20. 1 hit.
InterPro	IPR005031. Polyket_cyc. IPR023393. START-like_dom. [Graphical view]
Pfam	PF03364. Polyketide_cyc. 1 hit. [Graphical view]
ProtoNet	Search...
Other
GenomeRNAi	93058.
NextBio	77957.

Entry information

Entry name

CQ10A_HUMAN

Accession

Primary (citable) accession number: Q96MF6
Secondary accession number(s): Q6GMR6 , Q6UWB9, Q86X16, Q96MF1, Q9BUP4

Entry history

Integrated into UniProtKB/Swiss-Prot:	March 21, 2006
Last sequence update:	March 21, 2006
Last modified:	May 29, 2013
This is version 78 of the entry and version 2 of the sequence. [Complete history]

Entry status

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.