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Protein

Coenzyme Q-binding protein COQ10 homolog B, mitochondrial

Gene

COQ10B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 2 out of 5-Experimental evidence at transcript leveli

Functioni

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 (By similarity).By similarity

Names & Taxonomyi

Protein namesi
Recommended name:
Coenzyme Q-binding protein COQ10 homolog B, mitochondrial
Gene namesi
Name:COQ10B
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

HGNCiHGNC:25819. COQ10B.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA143485440.

Polymorphism and mutation databases

BioMutaiCOQ10B.
DMDMi74733854.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3737MitochondrionSequence analysisAdd
BLAST
Chaini38 – 238201Coenzyme Q-binding protein COQ10 homolog B, mitochondrialPRO_0000228647Add
BLAST

Proteomic databases

MaxQBiQ9H8M1.
PaxDbiQ9H8M1.
PRIDEiQ9H8M1.

PTM databases

iPTMnetiQ9H8M1.
PhosphoSiteiQ9H8M1.

Expressioni

Gene expression databases

BgeeiQ9H8M1.
CleanExiHS_COQ10B.
ExpressionAtlasiQ9H8M1. baseline and differential.
GenevisibleiQ9H8M1. HS.

Organism-specific databases

HPAiHPA046057.

Interactioni

Subunit structurei

Interacts with coenzyme Q.By similarity

Protein-protein interaction databases

BioGridi123186. 1 interaction.
IntActiQ9H8M1. 1 interaction.
STRINGi9606.ENSP00000263960.

Structurei

3D structure databases

ProteinModelPortaliQ9H8M1.
SMRiQ9H8M1. Positions 82-221.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the COQ10 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3177. Eukaryota.
COG2867. LUCA.
GeneTreeiENSGT00390000006193.
HOGENOMiHOG000261732.
HOVERGENiHBG081329.
InParanoidiQ9H8M1.
KOiK18588.
OMAiQMEYAFI.
OrthoDBiEOG7P2XT6.
PhylomeDBiQ9H8M1.
TreeFamiTF314447.

Family and domain databases

Gene3Di3.30.530.20. 1 hit.
InterProiIPR005031. COQ10_START.
IPR023393. START-like_dom.
[Graphical view]
PfamiPF03364. Polyketide_cyc. 1 hit.
[Graphical view]

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

Isoform 1 (identifier: Q9H8M1-1) [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
MAARTGHTAL RRVVSGCRPK SATAAGAQAP VRNGRYLASC GILMSRTLPL
60 70 80 90 100
HTSILPKEIC ARTFFKITAP LINKRKEYSE RRILGYSMQE MYDVVSGVED
110 120 130 140 150
YKHFVPWCKK SDVISKRSGY CKTRLEIGFP PVLERYTSVV TLVKPHLVKA
160 170 180 190 200
SCTDGRLFNH LETIWRFSPG LPGYPRTCTL DFSISFEFRS LLHSQLATLF
210 220 230
FDEVVKQMVA AFERRACKLY GPETNIPREL MLHEVHHT
Length:238
Mass (Da):27,071
Last modified:March 1, 2001 - v1
Checksum:i078ECF9A377908D0
GO
Isoform 2 (identifier: Q9H8M1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-43: Missing.

Note: No experimental confirmation available.
Show »
Length:195
Mass (Da):22,691
Checksum:iBE521AD970C147D5
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti48 – 481L → F.
Corresponds to variant rs34946819 [ dbSNP | Ensembl ].
VAR_033823

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 4343Missing in isoform 2. 1 PublicationVSP_056949Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK023510 mRNA. Translation: BAB14593.1.
AK294093 mRNA. Translation: BAH11670.1.
AC010746 Genomic DNA. No translation available.
BC022453 mRNA. Translation: AAH22453.1.
CCDSiCCDS2319.1. [Q9H8M1-1]
RefSeqiNP_001307747.1. NM_001320818.1.
NP_001307748.1. NM_001320819.1.
NP_001307749.1. NM_001320820.1.
NP_079423.1. NM_025147.4. [Q9H8M1-1]
UniGeneiHs.606592.

Genome annotation databases

EnsembliENST00000263960; ENSP00000263960; ENSG00000115520. [Q9H8M1-1]
GeneIDi80219.
KEGGihsa:80219.
UCSCiuc002uuh.2. human. [Q9H8M1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK023510 mRNA. Translation: BAB14593.1.
AK294093 mRNA. Translation: BAH11670.1.
AC010746 Genomic DNA. No translation available.
BC022453 mRNA. Translation: AAH22453.1.
CCDSiCCDS2319.1. [Q9H8M1-1]
RefSeqiNP_001307747.1. NM_001320818.1.
NP_001307748.1. NM_001320819.1.
NP_001307749.1. NM_001320820.1.
NP_079423.1. NM_025147.4. [Q9H8M1-1]
UniGeneiHs.606592.

3D structure databases

ProteinModelPortaliQ9H8M1.
SMRiQ9H8M1. Positions 82-221.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123186. 1 interaction.
IntActiQ9H8M1. 1 interaction.
STRINGi9606.ENSP00000263960.

PTM databases

iPTMnetiQ9H8M1.
PhosphoSiteiQ9H8M1.

Polymorphism and mutation databases

BioMutaiCOQ10B.
DMDMi74733854.

Proteomic databases

MaxQBiQ9H8M1.
PaxDbiQ9H8M1.
PRIDEiQ9H8M1.

Protocols and materials databases

DNASUi80219.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000263960; ENSP00000263960; ENSG00000115520. [Q9H8M1-1]
GeneIDi80219.
KEGGihsa:80219.
UCSCiuc002uuh.2. human. [Q9H8M1-1]

Organism-specific databases

CTDi80219.
GeneCardsiCOQ10B.
HGNCiHGNC:25819. COQ10B.
HPAiHPA046057.
neXtProtiNX_Q9H8M1.
PharmGKBiPA143485440.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3177. Eukaryota.
COG2867. LUCA.
GeneTreeiENSGT00390000006193.
HOGENOMiHOG000261732.
HOVERGENiHBG081329.
InParanoidiQ9H8M1.
KOiK18588.
OMAiQMEYAFI.
OrthoDBiEOG7P2XT6.
PhylomeDBiQ9H8M1.
TreeFamiTF314447.

Miscellaneous databases

ChiTaRSiCOQ10B. human.
GenomeRNAii80219.
NextBioi35478855.
PROiQ9H8M1.

Gene expression databases

BgeeiQ9H8M1.
CleanExiHS_COQ10B.
ExpressionAtlasiQ9H8M1. baseline and differential.
GenevisibleiQ9H8M1. HS.

Family and domain databases

Gene3Di3.30.530.20. 1 hit.
InterProiIPR005031. COQ10_START.
IPR023393. START-like_dom.
[Graphical view]
PfamiPF03364. Polyketide_cyc. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  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: Brain cortex and Placenta.
  2. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
    Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H.
    , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.
    Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  3. "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).
    Tissue: Brain.

Entry informationi

Entry nameiCQ10B_HUMAN
AccessioniPrimary (citable) accession number: Q9H8M1
Secondary accession number(s): B7Z1Y4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 21, 2006
Last sequence update: March 1, 2001
Last modified: May 11, 2016
This is version 95 of the entry and version 1 of the sequence. [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 chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.