COQ8B

Functionⁱ

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:24270420). Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway. Required for podocyte migration (PubMed:24270420).By similarity1 Publication

Activity regulationⁱ

Autoinhibited by the N-terminal domain, containing the KxGQ motif, that completely occludes the typical substrate binding pocket. Nucleotide-binding relieves inhibition.By similarity

Sites

Feature key	Position(s)	DescriptionActions	Length
Binding siteⁱ	219	ATPBy similarity	1
Binding siteⁱ	237	ATPBy similarity	1
Active siteⁱ	367	Proton acceptorBy similarity	1
Binding siteⁱ	372	ATPBy similarity	1
Binding siteⁱ	386	ATPBy similarity	1

Regions

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Nucleotide bindingⁱ	324 – 327	ATPBy similarity		4

GO - Molecular functionⁱ

ATP binding Source: UniProtKB-KW
kinase activity
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

cerebellar Purkinje cell layer morphogenesis
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 27499294
protein phosphorylation Source: GOC
ubiquinone biosynthetic process
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 27499294

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Kinase, Transferase
Ligand	ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCycⁱ	MetaCyc:ENSG00000123815-MONOMER

BioCycⁱ

MetaCyc:ENSG00000123815-MONOMER

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Atypical kinase COQ8B, mitochondrialCurated (EC:2.7.-.-By similarity) Alternative name(s): AarF domain-containing protein kinase 4Imported Coenzyme Q protein 8BCurated
Gene namesⁱ	Name:COQ8BImported Synonyms:ADCK4Imported
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 19

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000123815.11
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:19041 COQ8B
MIMⁱ	615567 gene
neXtProtⁱ	NX_Q96D53

Topology

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transmembraneⁱ	92 – 108	HelicalSequence analysisAdd BLAST		17

Keywords - Cellular componentⁱ

Cell membrane, Cytoplasm, Membrane, Mitochondrion

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Nephrotic syndrome 9 (NPHS9)2 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionA form of nephrotic syndrome, a renal disease clinically characterized by progressive renal failure, severe proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show focal segmental glomerulosclerosis.

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_076861	98	L → R in NPHS9. 1 Publication	1
Natural variantⁱVAR_070552	178	R → W in NPHS9. 2 PublicationsCorresponds to variant dbSNP:rs398122978EnsemblClinVar.	1
Natural variantⁱVAR_070553	286	D → G in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs398122979EnsemblClinVar.	1
Natural variantⁱVAR_076862	310	P → L in NPHS9. 1 Publication	1
Natural variantⁱVAR_070554	320	R → W in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs369573693EnsemblClinVar.	1
Natural variantⁱVAR_070555	343	R → W in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs398122981EnsemblClinVar.	1
Natural variantⁱVAR_070556	477	R → Q in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs1057519347Ensembl.	1
Natural variantⁱVAR_076863	498	A → E in NPHS9. 1 Publication	1

Keywords - Diseaseⁱ

Disease mutation

Organism-specific databases

DisGeNET More...DisGeNETⁱ	79934
MalaCards human disease database More...MalaCardsⁱ	COQ8B
MIMⁱ	615573 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000123815
Orphanetⁱ	93213 Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis
PharmGKBⁱ	PA134988974

Chemistry databases

ChEMBLⁱ	CHEMBL5753

ChEMBLⁱ

CHEMBL5753

Polymorphism and mutation databases

BioMutaⁱ	ADCK4
DMDMⁱ	74731415

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000271797	1 – 544	Atypical kinase COQ8B, mitochondrialAdd BLAST		544

Proteomic databases

EPDⁱ	Q96D53
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q96D53
PaxDbⁱ	Q96D53
PeptideAtlas More...PeptideAtlasⁱ	Q96D53
PRIDEⁱ	Q96D53
ProteomicsDBⁱ	76252 76253 [Q96D53-2]

PTM databases

iPTMnetⁱ	Q96D53
PhosphoSitePlusⁱ	Q96D53

Expressionⁱ

Tissue specificityⁱ

Widely expressed, including renal podocytes.1 Publication

Gene expression databases

Bgeeⁱ	ENSG00000123815 Expressed in 174 organ(s), highest expression level in adenohypophysis
CleanExⁱ	HS_ADCK4
ExpressionAtlasⁱ	Q96D53 baseline and differential
Genevisibleⁱ	Q96D53 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA027229 HPA027277 HPA027279 HPA028303

HPAⁱ

HPA027229
HPA027277
HPA027279
HPA028303

Interactionⁱ

Subunit structureⁱ

Homodimer; homodimerizes via its transmembrane region (PubMed:25216398). Interacts with COQ6 and COQ7 (PubMed:24270420). Interacts with the multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9 (PubMed:27499296).3 Publications

Protein-protein interaction databases

BioGridⁱ	123009, 36 interactors
IntActⁱ	Q96D53, 14 interactors
STRINGⁱ	9606.ENSP00000315118

Chemistry databases

BindingDBⁱ

Q96D53

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q96D53
SMRⁱ	Q96D53
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Domains and Repeats

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Domainⁱ	191 – 423	Protein kinaseAdd BLAST		233

Motif

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Motifⁱ	155 – 158	KxGQ motifBy similarity		4
Motifⁱ	216 – 219	AAAS motifBy similarity		4

Domainⁱ

Adopts an atypical protein kinase-like fold: while it adopts a core fold similar to that of well-characterized protein kinase-like domains. The KxGQ motif completely occludes the typical substrate binding pocket. Nucleotide-binding opens the substrate binding pocket and flips the active site from inside the hydrophobic core into a catalytically competent, solvent-exposed posture.By similarity

Sequence similaritiesⁱ

Belongs to the protein kinase superfamily. ADCK protein kinase family.Curated

Keywords - Domainⁱ

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGⁱ	KOG1234 Eukaryota COG0661 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00550000074739
HOGENOMⁱ	HOG000201140
HOVERGENⁱ	HBG061318
InParanoidⁱ	Q96D53
KEGG Orthology (KO) More...KOⁱ	K08869
OMAⁱ	ADYMPDW
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G02P9
PhylomeDBⁱ	Q96D53
TreeFamⁱ	TF300630

Family and domain databases

Conserved Domains Database More...CDDⁱ	cd13970 ABC1_ADCK3, 1 hit
InterProⁱ	View protein in InterPro IPR034646 ADCK3_UbiB IPR034638 COQ8B IPR011009 Kinase-like_dom_sf IPR004147 UbiB_dom
PANTHERⁱ	PTHR43851 PTHR43851, 1 hit PTHR43851:SF4 PTHR43851:SF4, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF03109 ABC1, 1 hit
SUPFAMⁱ	SSF56112 SSF56112, 1 hit

Sequences (2+)ⁱ

Sequence statusⁱ: Complete.

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

This entry has 2 described isoforms and 11 potential isoforms that are computationally mapped.Show all Align All

Isoform 1 (identifier: Q96D53-1) [UniParc]FASTA Add 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
MWLKVGGLLR GTGGQLGQTV GWPCGALGPG PHRWGPCGGS WAQKFYQDGP 
        60         70         80         90        100
GRGLGEEDIR RAREARPRKT PRPQLSDRSR ERKVPASRIS RLANFGGLAV 
       110        120        130        140        150
GLGLGVLAEM AKKSMPGGRL QSEGGSGLDS SPFLSEANAE RIVQTLCTVR 
       160        170        180        190        200
GAALKVGQML SIQDNSFISP QLQHIFERVR QSADFMPRWQ MLRVLEEELG 
       210        220        230        240        250
RDWQAKVASL EEVPFAAASI GQVHQGLLRD GTEVAVKIQY PGIAQSIQSD 
       260        270        280        290        300
VQNLLAVLKM SAALPAGLFA EQSLQALQQE LAWECDYRRE AACAQNFRQL 
       310        320        330        340        350
LANDPFFRVP AVVKELCTTR VLGMELAGGV PLDQCQGLSQ DLRNQICFQL 
       360        370        380        390        400
LTLCLRELFE FRFMQTDPNW ANFLYDASSH QVTLLDFGAS REFGTEFTDH 
       410        420        430        440        450
YIEVVKAAAD GDRDCVLQKS RDLKFLTGFE TKAFSDAHVE AVMILGEPFA 
       460        470        480        490        500
TQGPYDFGSG ETARRIQDLI PVLLRHRLCP PPEETYALHR KLAGAFLACA 
       510        520        530        540 
HLRAHIACRD LFQDTYHRYW ASRQPDAATA GSLPTKGDSW VDPS

Length:544

Mass (Da):60,069

Last modified:October 1, 2002 - v2

Checksum:ⁱ4645DF579B9DFA4B

GO

Isoform 2 (identifier: Q96D53-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
123-163: Missing.

« Hide

        10         20         30         40         50
MWLKVGGLLR GTGGQLGQTV GWPCGALGPG PHRWGPCGGS WAQKFYQDGP 
        60         70         80         90        100
GRGLGEEDIR RAREARPRKT PRPQLSDRSR ERKVPASRIS RLANFGGLAV 
       110        120        130        140        150
GLGLGVLAEM AKKSMPGGRL QSDNSFISPQ LQHIFERVRQ SADFMPRWQM 
       160        170        180        190        200
LRVLEEELGR DWQAKVASLE EVPFAAASIG QVHQGLLRDG TEVAVKIQYP 
       210        220        230        240        250
GIAQSIQSDV QNLLAVLKMS AALPAGLFAE QSLQALQQEL AWECDYRREA 
       260        270        280        290        300
ACAQNFRQLL ANDPFFRVPA VVKELCTTRV LGMELAGGVP LDQCQGLSQD 
       310        320        330        340        350
LRNQICFQLL TLCLRELFEF RFMQTDPNWA NFLYDASSHQ VTLLDFGASR 
       360        370        380        390        400
EFGTEFTDHY IEVVKAAADG DRDCVLQKSR DLKFLTGFET KAFSDAHVEA 
       410        420        430        440        450
VMILGEPFAT QGPYDFGSGE TARRIQDLIP VLLRHRLCPP PEETYALHRK 
       460        470        480        490        500
LAGAFLACAH LRAHIACRDL FQDTYHRYWA SRQPDAATAG SLPTKGDSWV 

DPS

Note: No experimental confirmation available.

Show »

Length:503

Mass (Da):55,853

Checksum:ⁱ547A81982FE22A6A

GO

Computationally mapped potential isoform sequencesⁱ

There are 11 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

	Entry	Entry name	Protein names	Gene names	Length	Annotation

	M0R011	M0R011_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	230	Annotation score:
	M0R3F7	M0R3F7_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	157	Annotation score:
	M0QZZ2	M0QZZ2_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	149	Annotation score:
	M0R340	M0R340_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	121	Annotation score:
	M0R0L2	M0R0L2_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	134	Annotation score:
	M0R307	M0R307_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	110	Annotation score:
	M0R001	M0R001_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	204	Annotation score:
	M0R362	M0R362_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	73	Annotation score:
	M0R0F4	M0R0F4_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	115	Annotation score:
	M0R2F4	M0R2F4_HUMAN	Atypical kinase COQ8B, mitochondria... Atypical kinase COQ8B, mitochondrial	COQ8B	81	Annotation score:
There is more potential isoformShow all

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	164 – 166	DNS → GTA in BAB14004 (PubMed:14702039).Curated		3

Natural variant

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_041420	78	R → C1 PublicationCorresponds to variant dbSNP:rs11538384EnsemblClinVar.	1
Natural variantⁱVAR_076861	98	L → R in NPHS9. 1 Publication	1
Natural variantⁱVAR_029995	174	H → R1 PublicationCorresponds to variant dbSNP:rs3865452EnsemblClinVar.	1
Natural variantⁱVAR_070552	178	R → W in NPHS9. 2 PublicationsCorresponds to variant dbSNP:rs398122978EnsemblClinVar.	1
Natural variantⁱVAR_070553	286	D → G in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs398122979EnsemblClinVar.	1
Natural variantⁱVAR_076862	310	P → L in NPHS9. 1 Publication	1
Natural variantⁱVAR_041421	318	T → M1 PublicationCorresponds to variant dbSNP:rs55899516Ensembl.	1
Natural variantⁱVAR_070554	320	R → W in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs369573693EnsemblClinVar.	1
Natural variantⁱVAR_070555	343	R → W in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs398122981EnsemblClinVar.	1
Natural variantⁱVAR_041422	352	T → R1 PublicationCorresponds to variant dbSNP:rs36012476EnsemblClinVar.	1
Natural variantⁱVAR_041423	462	T → M1 PublicationCorresponds to variant dbSNP:rs56083906Ensembl.	1
Natural variantⁱVAR_070556	477	R → Q in NPHS9. 1 PublicationCorresponds to variant dbSNP:rs1057519347Ensembl.	1
Natural variantⁱVAR_076863	498	A → E in NPHS9. 1 Publication	1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Alternative sequenceⁱVSP_022357	123 – 163	Missing in isoform 2. 1 PublicationAdd BLAST		41

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	BC013114 mRNA Translation: AAH13114.2 BC027473 mRNA Translation: AAH27473.1 AK022291 mRNA Translation: BAB14004.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS12562.1 [Q96D53-1] CCDS46081.1 [Q96D53-2]
NCBI Reference Sequences More...RefSeqⁱ	NP_001136027.1, NM_001142555.2 [Q96D53-2] NP_079152.3, NM_024876.3 [Q96D53-1]
UniGeneⁱ	Hs.130712

Genome annotation databases

Ensemblⁱ	ENST00000243583; ENSP00000243583; ENSG00000123815 [Q96D53-2] ENST00000324464; ENSP00000315118; ENSG00000123815 [Q96D53-1]
GeneIDⁱ	79934
KEGGⁱ	hsa:79934
UCSC genome browser More...UCSCⁱ	uc002ooq.3 human [Q96D53-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q96D53	Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		157	UniRef100_Q96D53
Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		204
Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		149
Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		110
Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		230
+1
Q96D53-2	AarF domain containing kinase 4, isoform CRA_b		HUMAN		503	UniRef100_A0A024R0Q9

Protein	Similar proteins		Species	Score	Length	Source
Q96D53	Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		157	UniRef90_Q96D53
Uncharacterized protein		RHIRO		637
Uncharacterized protein		CHLSB		544
Uncharacterized protein		NOMLE		544
Uncharacterized protein		COLAP		544
+45
Q96D53-2	AarF domain containing kinase 4, isoform CRA_b		HUMAN		503	UniRef90_A0A024R0Q9
Uncharacterized protein		AOTNA		504
Uncharacterized protein		CERAT		504
COQ8B isoform 3		PONAB		503
COQ8B isoform 3		PANTR		503
+24

Protein	Similar proteins		Species	Score	Length	Source
Q96D53	Atypical kinase COQ8B, mitochondrial (Fragment)		HUMAN		157	UniRef50_Q96D53
Uncharacterized protein		RHIRO		637
Uncharacterized protein		COLAP		544
aarF domain-containing protein kinase 4 isoform X1		TARSY		544
Putative aarF domain-containing protein kinase 4 isoform a		CALJA		544
+233

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	BC013114 mRNA Translation: AAH13114.2 BC027473 mRNA Translation: AAH27473.1 AK022291 mRNA Translation: BAB14004.1
CCDSⁱ	CCDS12562.1 [Q96D53-1] CCDS46081.1 [Q96D53-2]
RefSeqⁱ	NP_001136027.1, NM_001142555.2 [Q96D53-2] NP_079152.3, NM_024876.3 [Q96D53-1]
UniGeneⁱ	Hs.130712

3D structure databases

ProteinModelPortalⁱ	Q96D53
SMRⁱ	Q96D53
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	123009, 36 interactors
IntActⁱ	Q96D53, 14 interactors
STRINGⁱ	9606.ENSP00000315118

Chemistry databases

BindingDBⁱ	Q96D53
ChEMBLⁱ	CHEMBL5753

PTM databases

iPTMnetⁱ	Q96D53
PhosphoSitePlusⁱ	Q96D53

Polymorphism and mutation databases

BioMutaⁱ	ADCK4
DMDMⁱ	74731415

Proteomic databases

EPDⁱ	Q96D53
MaxQBⁱ	Q96D53
PaxDbⁱ	Q96D53
PeptideAtlasⁱ	Q96D53
PRIDEⁱ	Q96D53
ProteomicsDBⁱ	76252 76253 [Q96D53-2]

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	79934
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000243583; ENSP00000243583; ENSG00000123815 [Q96D53-2] ENST00000324464; ENSP00000315118; ENSG00000123815 [Q96D53-1]
GeneIDⁱ	79934
KEGGⁱ	hsa:79934
UCSCⁱ	uc002ooq.3 human [Q96D53-1]

Organism-specific databases

CTDⁱ	79934
DisGeNETⁱ	79934
EuPathDBⁱ	HostDB:ENSG00000123815.11
GeneCardsⁱ	COQ8B
HGNCⁱ	HGNC:19041 COQ8B
HPAⁱ	HPA027229 HPA027277 HPA027279 HPA028303
MalaCardsⁱ	COQ8B
MIMⁱ	615567 gene 615573 phenotype
neXtProtⁱ	NX_Q96D53
OpenTargetsⁱ	ENSG00000123815
Orphanetⁱ	93213 Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis
PharmGKBⁱ	PA134988974
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG1234 Eukaryota COG0661 LUCA
GeneTreeⁱ	ENSGT00550000074739
HOGENOMⁱ	HOG000201140
HOVERGENⁱ	HBG061318
InParanoidⁱ	Q96D53
KOⁱ	K08869
OMAⁱ	ADYMPDW
OrthoDBⁱ	EOG091G02P9
PhylomeDBⁱ	Q96D53
TreeFamⁱ	TF300630

Enzyme and pathway databases

BioCycⁱ	MetaCyc:ENSG00000123815-MONOMER

BioCycⁱ

MetaCyc:ENSG00000123815-MONOMER

Miscellaneous databases

ChiTaRSⁱ	COQ8B human
GenomeRNAiⁱ	79934
Protein Ontology More...PROⁱ	PR:Q96D53
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000123815 Expressed in 174 organ(s), highest expression level in adenohypophysis
CleanExⁱ	HS_ADCK4
ExpressionAtlasⁱ	Q96D53 baseline and differential
Genevisibleⁱ	Q96D53 HS

Family and domain databases

CDDⁱ	cd13970 ABC1_ADCK3, 1 hit
InterProⁱ	View protein in InterPro IPR034646 ADCK3_UbiB IPR034638 COQ8B IPR011009 Kinase-like_dom_sf IPR004147 UbiB_dom
PANTHERⁱ	PTHR43851 PTHR43851, 1 hit PTHR43851:SF4 PTHR43851:SF4, 1 hit
Pfamⁱ	View protein in Pfam PF03109 ABC1, 1 hit
SUPFAMⁱ	SSF56112 SSF56112, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	COQ8B_HUMAN
Accessionⁱ	Q96D53Primary (citable) accession number: Q96D53 Secondary accession number(s): Q8TAJ1, Q9HA52
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	January 9, 2007
	Last sequence update:	October 1, 2002
	Last modified:	November 7, 2018
	This is version 134 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	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.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
Human chromosome 19
Human chromosome 19: entries, gene names and cross-references to MIM
Human and mouse protein kinases
Human and mouse protein kinases: classification and index

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UniProtKB - Q96D53 (COQ8B_HUMAN)

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Atypical kinase COQ8B, mitochondrial

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Sites

Regions

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Mitochondrion

Cytosol

Plasma membrane

Cytosol

Mitochondrion

Plasma Membrane

Other locations

Topology

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Keywords - Diseasei

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

Chemistry databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Motif

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Experimental Info

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ