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Protein

Atypical kinase COQ8A, mitochondrial

Gene

Coq8a

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:27499294). Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:27499294). Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (By similarity). Shows an unusual selectivity for binding ADP over ATP (By similarity).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

Pathwayi: ubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.By similarity
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 sitei337ATPBy similarity1
Binding sitei355ATPBy similarity1
Active sitei485Proton acceptorBy similarity1
Binding sitei490ATPBy similarity1
Binding sitei504ATPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi442 – 445ATPBy similarity4

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionKinase, Transferase
Biological processUbiquinone biosynthesis
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

UniPathwayi
UPA00232

Names & Taxonomyi

Protein namesi
Recommended name:
Atypical kinase COQ8A, mitochondrialCurated (EC:2.7.-.-By similarity)
Alternative name(s):
Chaperone activity of bc1 complex-likeBy similarity
Short name:
Chaperone-ABC1-likeBy similarity
Coenzyme Q protein 8ABy similarity
aarF domain-containing protein kinase 3Imported
Gene namesi
Name:Coq8aBy similarity
Synonyms:Adck3Imported, Cabc1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 1

Organism-specific databases

MGIiMGI:1914676 Coq8a

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei211 – 227HelicalSequence analysisAdd BLAST17

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Disruption phenotypei

Mice were born at the expected Mendelian frequency and do not show overt phenotype under normal conditions. They however develop a slowly progressive loss of coordination after birth and develop ataxia and seizures. Defects are due to dysfunctional cerebellar Purkinje cells and defective skeletal muscle. Mice display tissue-specific coenzyme Q deficiency: coenzyme Q levels are normal in younger mice but significantly and specifically reduced in skeletal muscle. However, normal coenzyme Q levels are observed in whole cerebella, suggesting that cerebellar Purkinje cells are specifically affected.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 159MitochondrionBy similarityAdd BLAST159
ChainiPRO_0000000263160 – 645Atypical kinase COQ8A, mitochondrialAdd BLAST486

Proteomic databases

PaxDbiQ60936
PeptideAtlasiQ60936
PRIDEiQ60936

PTM databases

iPTMnetiQ60936
PhosphoSitePlusiQ60936
SwissPalmiQ60936

Expressioni

Tissue specificityi

Present in various tissues (at protein level).1 Publication

Gene expression databases

BgeeiENSMUSG00000026489 Expressed in 256 organ(s), highest expression level in pituitary gland
ExpressionAtlasiQ60936 baseline and differential
GenevisibleiQ60936 MM

Interactioni

Subunit structurei

Homodimer; homodimerizes via its transmembrane region. Interacts with the multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9.By similarity

Protein-protein interaction databases

BioGridi212178, 1 interactor
IntActiQ60936, 4 interactors
MINTiQ60936
STRINGi10090.ENSMUSP00000027766

Structurei

3D structure databases

ProteinModelPortaliQ60936
SMRiQ60936
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini326 – 515Protein kinaseAdd BLAST190

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi273 – 276KxGQ motifBy similarity4
Motifi334 – 337AAAS motifBy similarity4

Domaini

Adopts an atypical protein kinase-like fold: while it adopts a core fold similar to that of well-characterized protein kinase-like domains, a number of features are positioned to inhibit the kinase activity: (1) an atypical AAAS motif in an alanine-rich (A-rich) loop that replaces the canonical glycine-rich (G-rich) nucleotide-binding loop and limits ATP binding by establishing an unusual selectivity for ADP and (2) an N-terminal domain, containing the KxGQ motif, that 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

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1234 Eukaryota
COG0661 LUCA
GeneTreeiENSGT00550000074739
HOGENOMiHOG000201140
HOVERGENiHBG061318
InParanoidiQ60936
KOiK08869
OMAiKYWEGRT
OrthoDBiEOG091G02P9
PhylomeDBiQ60936
TreeFamiTF300630

Family and domain databases

CDDicd13970 ABC1_ADCK3, 1 hit
InterProiView protein in InterPro
IPR034646 ADCK3_UbiB
IPR034640 COQ8A
IPR011009 Kinase-like_dom_sf
IPR004147 UbiB_dom
PANTHERiPTHR43851 PTHR43851, 1 hit
PTHR43851:SF1 PTHR43851:SF1, 1 hit
PfamiView protein in Pfam
PF03109 ABC1, 1 hit
SUPFAMiSSF56112 SSF56112, 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 14 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q60936-1) [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
MAAMLGDAIM VAKGLAKLTQ AAVETHLQNL GLGGELLLAA RALQSTAVEQ
60 70 80 90 100
FSMVFGKVQG QDKHEDSYAT ENFEDLEAEV QFSTPQAAGT SLDFSAASSL
110 120 130 140 150
DQSLSPSHSQ GPAPAYASSG PFREAGLPGQ ATSPMGRVNG RLFVDHRDLF
160 170 180 190 200
LANGIQRRSF HQDQSSVGGL TAEDIEKARQ AKARPESKPH KQMLSERARE
210 220 230 240 250
RKVPVTRIGR LANFGGLAVG LGIGALAEVA KKSLRSENST GKKAVLDSSP
260 270 280 290 300
FLSEANAERI VSTLCKVRGA ALKLGQMLSI QDDAFINPHL AKIFERVRQS
310 320 330 340 350
ADFMPLKQMT KTLNSDLGPH WRDKLEYFEE RPFAAASIGQ VHLARMKGGR
360 370 380 390 400
EVAMKIQYPG VAQSINSDVN NLMAVLNMSN MLPEGLFPEH LIDVLRRELT
410 420 430 440 450
LECDYQREAA YAKKFRELLK DHPFFYVPEI VDELCSPHVL TTELISGFPL
460 470 480 490 500
DQAEGLSQEV RNEICYNILV LCLRELFEFH VMQTDPNWSN FFYDPQQHKV
510 520 530 540 550
ALLDFGATRE YDRSFTDLYI QVIRAAADQD REAVLKKSIE MKFLTGYEVK
560 570 580 590 600
AMEDAHLDAI LILGEAFASE EPFDFGTQST TEKIHNLIPV MLKHRLIPPP
610 620 630 640
EETYSLHRKM GGSFLICSKL KARFPCKAMF EEAYSNYCRM KSGLQ
Length:645
Mass (Da):71,743
Last modified:January 27, 2003 - v2
Checksum:iC94854B41211F880
GO
Isoform 2 (identifier: Q60936-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-276: Missing.

Note: No experimental confirmation available.
Show »
Length:369
Mass (Da):42,564
Checksum:iE4CB1DDCC02B4309
GO

Computationally mapped potential isoform sequencesi

There are 14 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F7B1B6F7B1B6_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
401Annotation score:
E0CYT4E0CYT4_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
71Annotation score:
E0CXR1E0CXR1_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
139Annotation score:
E0CYG7E0CYG7_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
71Annotation score:
E0CYD3E0CYD3_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
70Annotation score:
E0CXT4E0CXT4_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
108Annotation score:
F6T0A8F6T0A8_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
149Annotation score:
E0CYC1E0CYC1_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
130Annotation score:
A0A0A6YXI0A0A0A6YXI0_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
60Annotation score:
E0CXR6E0CXR6_MOUSE
Atypical kinase COQ8A, mitochondria...
Coq8a Adck3
117Annotation score:
There are more potential isoformsShow all

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti213N → D in BAB29459 (PubMed:16141072).Curated1
Sequence conflicti388P → S in BAB29459 (PubMed:16141072).Curated1
Sequence conflicti546 – 547GY → RP in AAA86413 (Ref. 4) Curated2

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0223541 – 276Missing in isoform 2. 1 PublicationAdd BLAST276

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ278735 mRNA Translation: CAC00538.1
AK004791 mRNA Translation: BAB23567.2
AK014605 mRNA Translation: BAB29459.2
U31629 mRNA Translation: AAA86413.1
CCDSiCCDS15566.1 [Q60936-1]
RefSeqiNP_001156762.1, NM_001163290.1 [Q60936-1]
NP_075830.2, NM_023341.3 [Q60936-1]
XP_006497024.1, XM_006496961.2 [Q60936-1]
XP_006497025.1, XM_006496962.2 [Q60936-1]
UniGeneiMm.38330

Genome annotation databases

EnsembliENSMUST00000027766; ENSMUSP00000027766; ENSMUSG00000026489 [Q60936-1]
ENSMUST00000170472; ENSMUSP00000128290; ENSMUSG00000026489 [Q60936-1]
GeneIDi67426
KEGGimmu:67426
UCSCiuc007dvx.2 mouse [Q60936-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ278735 mRNA Translation: CAC00538.1
AK004791 mRNA Translation: BAB23567.2
AK014605 mRNA Translation: BAB29459.2
U31629 mRNA Translation: AAA86413.1
CCDSiCCDS15566.1 [Q60936-1]
RefSeqiNP_001156762.1, NM_001163290.1 [Q60936-1]
NP_075830.2, NM_023341.3 [Q60936-1]
XP_006497024.1, XM_006496961.2 [Q60936-1]
XP_006497025.1, XM_006496962.2 [Q60936-1]
UniGeneiMm.38330

3D structure databases

ProteinModelPortaliQ60936
SMRiQ60936
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi212178, 1 interactor
IntActiQ60936, 4 interactors
MINTiQ60936
STRINGi10090.ENSMUSP00000027766

PTM databases

iPTMnetiQ60936
PhosphoSitePlusiQ60936
SwissPalmiQ60936

Proteomic databases

PaxDbiQ60936
PeptideAtlasiQ60936
PRIDEiQ60936

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000027766; ENSMUSP00000027766; ENSMUSG00000026489 [Q60936-1]
ENSMUST00000170472; ENSMUSP00000128290; ENSMUSG00000026489 [Q60936-1]
GeneIDi67426
KEGGimmu:67426
UCSCiuc007dvx.2 mouse [Q60936-1]

Organism-specific databases

CTDi56997
MGIiMGI:1914676 Coq8a

Phylogenomic databases

eggNOGiKOG1234 Eukaryota
COG0661 LUCA
GeneTreeiENSGT00550000074739
HOGENOMiHOG000201140
HOVERGENiHBG061318
InParanoidiQ60936
KOiK08869
OMAiKYWEGRT
OrthoDBiEOG091G02P9
PhylomeDBiQ60936
TreeFamiTF300630

Enzyme and pathway databases

UniPathwayi
UPA00232

Miscellaneous databases

ChiTaRSiCoq8a mouse
PROiPR:Q60936
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000026489 Expressed in 256 organ(s), highest expression level in pituitary gland
ExpressionAtlasiQ60936 baseline and differential
GenevisibleiQ60936 MM

Family and domain databases

CDDicd13970 ABC1_ADCK3, 1 hit
InterProiView protein in InterPro
IPR034646 ADCK3_UbiB
IPR034640 COQ8A
IPR011009 Kinase-like_dom_sf
IPR004147 UbiB_dom
PANTHERiPTHR43851 PTHR43851, 1 hit
PTHR43851:SF1 PTHR43851:SF1, 1 hit
PfamiView protein in Pfam
PF03109 ABC1, 1 hit
SUPFAMiSSF56112 SSF56112, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCOQ8A_MOUSE
AccessioniPrimary (citable) accession number: Q60936
Secondary accession number(s): Q8K0M1
, Q9D657, Q9DBR5, Q9JHR3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 27, 2003
Last modified: November 7, 2018
This is version 133 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. Human and mouse protein kinases
    Human and mouse protein kinases: classification and index
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