Q5HYK3 (COQ5_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial
EC=2.1.1.201

Alternative name(s):
Ubiquinone biosynthesis methyltransferase COQ5

Gene names

Name:

COQ5

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

General annotation (Comments)

Function	Methyltransferase required for the conversion of 2-polyprenyl-6-methoxy-1,4-benzoquinol (DDMQH2) to 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) By similarity.
Catalytic activity	S-adenosyl-L-methionine + 2-methoxy-6-all-trans-polyprenyl-1,4-benzoquinol = S-adenosyl-L-homocysteine + 6-methoxy-3-methyl-2-all-trans-polyprenyl-1,4-benzoquinol.
Pathway	Cofactor biosynthesis; ubiquinone biosynthesis.
Subcellular location	Mitochondrion By similarity.
Sequence similarities	Belongs to the methyltransferase superfamily. UbiE family.

Ontologies

Keywords
Biological process	Ubiquinone biosynthesis
Cellular component	Mitochondrion
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Transit peptide
Molecular function	Methyltransferase Transferase
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	ubiquinone biosynthetic process Inferred from electronic annotation. Source: UniProtKB-UniPathway
Cellular_component	mitochondrion Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular_function	methyltransferase activity Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Alternative products

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

Isoform 1 (identifier: Q5HYK3-1)

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: Q5HYK3-2)

The sequence of this isoform differs from the canonical sequence as follows:
     119-170: Missing.
     189-327: YRAGLAWVLG...IVAIHSGFKL → HDRRCRLSQG...GIWSVSLLNC

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 49	49	Mitochondrion Potential
	Chain	50 – 327	278	2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial		PRO_0000228628
Natural variations
	Alternative sequence	119 – 170	52	Missing in isoform 2.		VSP_017681
	Alternative sequence	189 – 327	139	YRAGL…SGFKL → HDRRCRLSQGDLRKSNIRHC GHSFWLQTLIPFLSWSMNQS YPVESLELKDNLANETAAEH LLLRIRALDSCLNRTVSKWK NKFLSLFYSFLWSCFSPSPR GIWSVSLLNC in isoform 2.		VSP_017682
	Natural variant	152	1	A → T. Ref.2 Ref.5 Corresponds to variant rs3742049 [ dbSNP | Ensembl ].		VAR_025702
Experimental info
	Sequence conflict	78	1	K → R in CAI46073. Ref.3
	Sequence conflict	291	1	F → S in AAH04916. Ref.5

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified March 21, 2006. Version 2. Checksum: 7D601DF6E74FFCC4	FASTA	327	37,140
	10 20 30 40 50 60 MAAPGSCALW SYCGRGWSRA MRGCQLLGLR SSWPGDLLSA RLLSQEKRAA ETHFGFETVS 70 80 90 100 110 120 EEEKGGKVYQ VFESVAKKYD VMNDMMSLGI HRVWKDLLLW KMHPLPGTQL LDVAGGTGDI 130 140 150 160 170 180 AFRFLNYVQS QHQRKQKRQL RAQQNLSWEE IAKEYQNEED SLGGSRVVVC DINKEMLKVG 190 200 210 220 230 240 KQKALAQGYR AGLAWVLGDA EELPFDDDKF DIYTIAFGIR NVTHIDQALQ EAHRVLKPGG 250 260 270 280 290 300 RFLCLEFSQV NNPLISRLYD LYSFQVIPVL GEVIAGDWKS YQYLVESIRR FPSQEEFKDM 310 320 IEDAGFHKVT YESLTSGIVA IHSGFKL
	Isoform 2 [UniParc]. Checksum: FBE03D6ED4624CF9	FASTA	246	27,914
	10 20 30 40 50 60 MAAPGSCALW SYCGRGWSRA MRGCQLLGLR SSWPGDLLSA RLLSQEKRAA ETHFGFETVS 70 80 90 100 110 120 EEEKGGKVYQ VFESVAKKYD VMNDMMSLGI HRVWKDLLLW KMHPLPGTQL LDVAGGTGDI 130 140 150 160 170 180 NKEMLKVGKQ KALAQGHDRR CRLSQGDLRK SNIRHCGHSF WLQTLIPFLS WSMNQSYPVE 190 200 210 220 230 240 SLELKDNLAN ETAAEHLLLR IRALDSCLNR TVSKWKNKFL SLFYSFLWSC FSPSPRGIWS VSLLNC

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: Cerebellum.
[2]	Suzuki Y., Sugano S., Totoki Y., Toyoda A., Takeda T., Sakaki Y., Tanaka A., Yokoyama S. Submitted (APR-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), VARIANT THR-152. Tissue: Coronary artery.
[3]	"The full-ORF clone resource of the German cDNA consortium." Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U., Wellenreuther R., Mehrle A., Schuster C., Bahr A., Bloecker H., Heubner D., Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A., Wiemann S., Schupp I. BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1). Tissue: Adipose tissue.
[4]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	"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 THR-152. Tissue: Lung.
[6]	"Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AK057777 mRNA. Translation: BAB71567.1. AK222610 mRNA. Translation: BAD96330.1. AK293656 mRNA. Translation: BAG57105.1. BX647562 mRNA. Translation: CAI46073.1. CH471054 Genomic DNA. Translation: EAW98198.1. BC004916 mRNA. Translation: AAH04916.2. BC107874 mRNA. Translation: AAI07875.1.
IPI	IPI00456965. IPI00742661.
RefSeq	NP_115690.3. NM_032314.3.
UniGene	Hs.17250.
3D structure databases
ProteinModelPortal	Q5HYK3.
ModBase	Search...
Protein-protein interaction databases
STRING	9606.ENSP00000288532.
PTM databases
PhosphoSite	Q5HYK3.
Polymorphism databases
DMDM	90111987.
Proteomic databases
PaxDb	Q5HYK3.
PRIDE	Q5HYK3.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000288532; ENSP00000288532; ENSG00000110871.
GeneID	84274.
KEGG	hsa:84274.
UCSC	uc001tyn.3. human.
Organism-specific databases
CTD	84274.
GeneCards	GC12M120941.
HGNC	HGNC:28722. COQ5.
HPA	HPA049487.
neXtProt	NX_Q5HYK3.
PharmGKB	PA143485438.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG2226.
HOGENOM	HOG000249463.
HOVERGEN	HBG081303.
InParanoid	Q5HYK3.
KO	K06127.
OMA	PYQYLVE.
Enzyme and pathway databases
UniPathway	UPA00232.
Gene expression databases
ArrayExpress	Q5HYK3.
Bgee	Q5HYK3.
CleanEx	HS_COQ5.
Genevestigator	Q5HYK3.
GermOnline	ENSG00000110871. Homo sapiens.
Family and domain databases
InterPro	IPR004033. UbiE/COQ5_MeTrFase. IPR023576. UbiE/COQ5_MeTrFase_CS. IPR025765. UbiE/COQ5_MeTrFase_dom. [Graphical view]
PANTHER	PTHR10108:SF24. PTHR10108:SF24. 1 hit.
Pfam	PF01209. Ubie_methyltran. 1 hit. [Graphical view]
TIGRFAMs	TIGR01934. MenG_MenH_UbiE. 1 hit.
PROSITE	PS01183. UBIE_1. 1 hit. PS01184. UBIE_2. 1 hit. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	COQ5. human.
GenomeRNAi	84274.
NextBio	73846.

Entry information

Entry name

COQ5_HUMAN

Accession

Primary (citable) accession number: Q5HYK3
Secondary accession number(s): B4DEJ4 , Q32Q28, Q53HH0, Q96LV1, Q9BSP8

Entry history

Integrated into UniProtKB/Swiss-Prot:	March 21, 2006
Last sequence update:	March 21, 2006
Last modified:	May 1, 2013
This is version 82 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.