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Protein

2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial

Gene

COQ5

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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).UniRule annotation

Catalytic activityi

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.UniRule annotation

Pathwayi: ubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.UniRule annotation1 Publication
View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei117S-adenosyl-L-methionineUniRule annotation1
Binding sitei171S-adenosyl-L-methionineUniRule annotation1

GO - Molecular functioni

GO - Biological processi

  • methylation Source: UniProtKB
  • ubiquinone biosynthetic process Source: UniProtKB

Keywordsi

Molecular functionMethyltransferase, Transferase
Biological processUbiquinone biosynthesis
LigandS-adenosyl-L-methionine

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000110871-MONOMER
ReactomeiR-HSA-2142789 Ubiquinol biosynthesis
UniPathwayi
UPA00232

Names & Taxonomyi

Protein namesi
Recommended name:
2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrialUniRule annotation (EC:2.1.1.201UniRule annotation)
Alternative name(s):
Ubiquinone biosynthesis methyltransferase COQ5UniRule annotation
Gene namesi
Name:COQ5UniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

EuPathDBiHostDB:ENSG00000110871.14
HGNCiHGNC:28722 COQ5
MIMi616359 gene
neXtProtiNX_Q5HYK3

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

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi84274
OpenTargetsiENSG00000110871
PharmGKBiPA143485438

Polymorphism and mutation databases

BioMutaiCOQ5
DMDMi90111987

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 42MitochondrionUniRule annotationAdd BLAST42
ChainiPRO_000022862843 – 3272-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrialAdd BLAST285

Proteomic databases

EPDiQ5HYK3
MaxQBiQ5HYK3
PaxDbiQ5HYK3
PeptideAtlasiQ5HYK3
PRIDEiQ5HYK3
ProteomicsDBi62942
62943 [Q5HYK3-2]

PTM databases

iPTMnetiQ5HYK3
PhosphoSitePlusiQ5HYK3

Expressioni

Tissue specificityi

Widely expressed, with highest levels in liver, lung, placenta and skeletal muscle.1 Publication

Gene expression databases

BgeeiENSG00000110871 Expressed in 99 organ(s), highest expression level in muscle of leg
CleanExiHS_COQ5
ExpressionAtlasiQ5HYK3 baseline and differential
GenevisibleiQ5HYK3 HS

Organism-specific databases

HPAiHPA049487

Interactioni

Subunit structurei

Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9.UniRule annotation1 Publication

Binary interactionsi

Protein-protein interaction databases

BioGridi124001, 29 interactors
IntActiQ5HYK3, 13 interactors
STRINGi9606.ENSP00000288532

Structurei

3D structure databases

ProteinModelPortaliQ5HYK3
SMRiQ5HYK3
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni199 – 200S-adenosyl-L-methionine bindingUniRule annotation2

Sequence similaritiesi

Belongs to the class I-like SAM-binding methyltransferase superfamily. MenG/UbiE family.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1540 Eukaryota
COG2226 LUCA
GeneTreeiENSGT00390000001654
HOGENOMiHOG000249463
HOVERGENiHBG081303
InParanoidiQ5HYK3
KOiK06127
OMAiVRNFENL
OrthoDBiEOG091G0G2Z
PhylomeDBiQ5HYK3
TreeFamiTF106217

Family and domain databases

HAMAPiMF_01813 MenG_UbiE_methyltr, 1 hit
InterProiView protein in InterPro
IPR029063 SAM-dependent_MTases
IPR004033 UbiE/COQ5_MeTrFase
IPR023576 UbiE/COQ5_MeTrFase_CS
PfamiView protein in Pfam
PF01209 Ubie_methyltran, 1 hit
SUPFAMiSSF53335 SSF53335, 2 hits
TIGRFAMsiTIGR01934 MenG_MenH_UbiE, 1 hit
PROSITEiView protein in PROSITE
PS51608 SAM_MT_UBIE, 1 hit
PS01183 UBIE_1, 1 hit
PS01184 UBIE_2, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q5HYK3-1) [UniParc]FASTAAdd to basket

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.

« Hide
        10         20         30         40         50
MAAPGSCALW SYCGRGWSRA MRGCQLLGLR SSWPGDLLSA RLLSQEKRAA
60 70 80 90 100
ETHFGFETVS EEEKGGKVYQ VFESVAKKYD VMNDMMSLGI HRVWKDLLLW
110 120 130 140 150
KMHPLPGTQL LDVAGGTGDI AFRFLNYVQS QHQRKQKRQL RAQQNLSWEE
160 170 180 190 200
IAKEYQNEED SLGGSRVVVC DINKEMLKVG KQKALAQGYR AGLAWVLGDA
210 220 230 240 250
EELPFDDDKF DIYTIAFGIR NVTHIDQALQ EAHRVLKPGG RFLCLEFSQV
260 270 280 290 300
NNPLISRLYD LYSFQVIPVL GEVIAGDWKS YQYLVESIRR FPSQEEFKDM
310 320
IEDAGFHKVT YESLTSGIVA IHSGFKL
Length:327
Mass (Da):37,140
Last modified:March 21, 2006 - v2
Checksum:i7D601DF6E74FFCC4
GO
Isoform 2 (identifier: Q5HYK3-2) [UniParc]FASTAAdd to basket

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.
Show »
Length:246
Mass (Da):27,914
Checksum:iFBE03D6ED4624CF9
GO

Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
B4DP72B4DP72_HUMAN
2-methoxy-6-polyprenyl-1,4-benzoqui...
COQ5
253Annotation score:
F8VVX6F8VVX6_HUMAN
2-methoxy-6-polyprenyl-1,4-benzoqui...
COQ5
213Annotation score:
F8VVD5F8VVD5_HUMAN
2-methoxy-6-polyprenyl-1,4-benzoqui...
COQ5
91Annotation score:
F8VP53F8VP53_HUMAN
2-methoxy-6-polyprenyl-1,4-benzoqui...
COQ5
130Annotation score:
F8VVW7F8VVW7_HUMAN
2-methoxy-6-polyprenyl-1,4-benzoqui...
COQ5
176Annotation score:

Sequence cautioni

The sequence BAG38046 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti78K → R in CAI46073 (PubMed:17974005).Curated1
Sequence conflicti171D → G in BAG38046 (PubMed:14702039).Curated1
Sequence conflicti291F → S in AAH04916 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_025702152A → T3 PublicationsCorresponds to variant dbSNP:rs3742049Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_017681119 – 170Missing in isoform 2. 1 PublicationAdd BLAST52
Alternative sequenceiVSP_017682189 – 327YRAGL…SGFKL → HDRRCRLSQGDLRKSNIRHC GHSFWLQTLIPFLSWSMNQS YPVESLELKDNLANETAAEH LLLRIRALDSCLNRTVSKWK NKFLSLFYSFLWSCFSPSPR GIWSVSLLNC in isoform 2. 1 PublicationAdd BLAST139

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
EU700459 mRNA Translation: ACD75052.1
AK057777 mRNA Translation: BAB71567.1
AK222610 mRNA Translation: BAD96330.1
AK293656 mRNA Translation: BAG57105.1
AK315681 mRNA Translation: BAG38046.1 Different initiation.
BX647562 mRNA Translation: CAI46073.1
CH471054 Genomic DNA Translation: EAW98198.1
BC004916 mRNA Translation: AAH04916.2
BC107874 mRNA Translation: AAI07875.1
CCDSiCCDS31912.1 [Q5HYK3-1]
RefSeqiNP_115690.3, NM_032314.3 [Q5HYK3-1]
UniGeneiHs.17250

Genome annotation databases

EnsembliENST00000288532; ENSP00000288532; ENSG00000110871 [Q5HYK3-1]
GeneIDi84274
KEGGihsa:84274
UCSCiuc001tyn.4 human [Q5HYK3-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
EU700459 mRNA Translation: ACD75052.1
AK057777 mRNA Translation: BAB71567.1
AK222610 mRNA Translation: BAD96330.1
AK293656 mRNA Translation: BAG57105.1
AK315681 mRNA Translation: BAG38046.1 Different initiation.
BX647562 mRNA Translation: CAI46073.1
CH471054 Genomic DNA Translation: EAW98198.1
BC004916 mRNA Translation: AAH04916.2
BC107874 mRNA Translation: AAI07875.1
CCDSiCCDS31912.1 [Q5HYK3-1]
RefSeqiNP_115690.3, NM_032314.3 [Q5HYK3-1]
UniGeneiHs.17250

3D structure databases

ProteinModelPortaliQ5HYK3
SMRiQ5HYK3
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124001, 29 interactors
IntActiQ5HYK3, 13 interactors
STRINGi9606.ENSP00000288532

PTM databases

iPTMnetiQ5HYK3
PhosphoSitePlusiQ5HYK3

Polymorphism and mutation databases

BioMutaiCOQ5
DMDMi90111987

Proteomic databases

EPDiQ5HYK3
MaxQBiQ5HYK3
PaxDbiQ5HYK3
PeptideAtlasiQ5HYK3
PRIDEiQ5HYK3
ProteomicsDBi62942
62943 [Q5HYK3-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000288532; ENSP00000288532; ENSG00000110871 [Q5HYK3-1]
GeneIDi84274
KEGGihsa:84274
UCSCiuc001tyn.4 human [Q5HYK3-1]

Organism-specific databases

CTDi84274
DisGeNETi84274
EuPathDBiHostDB:ENSG00000110871.14
GeneCardsiCOQ5
HGNCiHGNC:28722 COQ5
HPAiHPA049487
MIMi616359 gene
neXtProtiNX_Q5HYK3
OpenTargetsiENSG00000110871
PharmGKBiPA143485438
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1540 Eukaryota
COG2226 LUCA
GeneTreeiENSGT00390000001654
HOGENOMiHOG000249463
HOVERGENiHBG081303
InParanoidiQ5HYK3
KOiK06127
OMAiVRNFENL
OrthoDBiEOG091G0G2Z
PhylomeDBiQ5HYK3
TreeFamiTF106217

Enzyme and pathway databases

UniPathwayi
UPA00232

BioCyciMetaCyc:ENSG00000110871-MONOMER
ReactomeiR-HSA-2142789 Ubiquinol biosynthesis

Miscellaneous databases

ChiTaRSiCOQ5 human
GenomeRNAii84274
PROiPR:Q5HYK3
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000110871 Expressed in 99 organ(s), highest expression level in muscle of leg
CleanExiHS_COQ5
ExpressionAtlasiQ5HYK3 baseline and differential
GenevisibleiQ5HYK3 HS

Family and domain databases

HAMAPiMF_01813 MenG_UbiE_methyltr, 1 hit
InterProiView protein in InterPro
IPR029063 SAM-dependent_MTases
IPR004033 UbiE/COQ5_MeTrFase
IPR023576 UbiE/COQ5_MeTrFase_CS
PfamiView protein in Pfam
PF01209 Ubie_methyltran, 1 hit
SUPFAMiSSF53335 SSF53335, 2 hits
TIGRFAMsiTIGR01934 MenG_MenH_UbiE, 1 hit
PROSITEiView protein in PROSITE
PS51608 SAM_MT_UBIE, 1 hit
PS01183 UBIE_1, 1 hit
PS01184 UBIE_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCOQ5_HUMAN
AccessioniPrimary (citable) accession number: Q5HYK3
Secondary accession number(s): B2RDU9
, B3GK62, B4DEJ4, Q32Q28, Q53HH0, Q96LV1, Q9BSP8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 21, 2006
Last sequence update: March 21, 2006
Last modified: October 10, 2018
This is version 131 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

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. SIMILARITY comments
    Index of protein domains and families
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  5. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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