Atypical kinase COQ8B, mitochondrial

Details

Name
Atypical kinase COQ8B, mitochondrial
Synonyms
  • 2.7.-.-
  • AarF domain-containing protein kinase 4
  • ADCK4
  • Coenzyme Q protein 8B
Gene Name
COQ8B
Organism
Humans
Amino acid sequence
>lcl|BSEQ0051698|Atypical kinase COQ8B, mitochondrial
MWLKVGGLLRGTGGQLGQTVGWPCGALGPGPHRWGPCGGSWAQKFYQDGPGRGLGEEDIR
RAREARPRKTPRPQLSDRSRERKVPASRISRLANFGGLAVGLGLGVLAEMAKKSMPGGRL
QSEGGSGLDSSPFLSEANAERIVQTLCTVRGAALKVGQMLSIQDNSFISPQLQHIFERVR
QSADFMPRWQMLRVLEEELGRDWQAKVASLEEVPFAAASIGQVHQGLLRDGTEVAVKIQY
PGIAQSIQSDVQNLLAVLKMSAALPAGLFAEQSLQALQQELAWECDYRREAACAQNFRQL
LANDPFFRVPAVVKELCTTRVLGMELAGGVPLDQCQGLSQDLRNQICFQLLTLCLRELFE
FRFMQTDPNWANFLYDASSHQVTLLDFGASREFGTEFTDHYIEVVKAAADGDRDCVLQKS
RDLKFLTGFETKAFSDAHVEAVMILGEPFATQGPYDFGSGETARRIQDLIPVLLRHRLCP
PPEETYALHRKLAGAFLACAHLRAHIACRDLFQDTYHRYWASRQPDAATAGSLPTKGDSW
VDPS
Number of residues
544
Molecular Weight
60068.925
Theoretical pI
Not Available
GO Classification
Functions
ATP binding / kinase activity
Processes
cerebellar Purkinje cell layer morphogenesis / ubiquinone biosynthetic process
Components
cytosol / integral component of membrane / mitochondrial membrane / mitochondrion / plasma membrane
General Function
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).
Specific Function
Atp binding
Pfam Domain Function
Transmembrane Regions
92-108
Cellular Location
Mitochondrion membrane
Gene sequence
>lcl|BSEQ0051699|Atypical kinase COQ8B, mitochondrial (COQ8B)
ATGTGGCTGAAGGTGGGGGGCCTACTTCGGGGGACCGGTGGACAGCTGGGCCAGACTGTT
GGTTGGCCTTGTGGGGCCCTGGGGCCTGGGCCCCACCGCTGGGGACCATGTGGAGGTTCT
TGGGCCCAAAAGTTTTACCAGGATGGGCCTGGGAGAGGCCTGGGTGAGGAGGACATTCGC
AGGGCACGGGAGGCCCGTCCCAGGAAGACACCCCGGCCCCAGCTGAGTGACCGCTCTCGA
GAACGCAAGGTGCCTGCCTCCCGCATCAGCCGCTTGGCCAACTTTGGGGGACTGGCTGTG
GGCTTGGGGCTAGGAGTACTGGCCGAGATGGCTAAGAAGTCCATGCCAGGAGGTCGTCTG
CAGTCAGAGGGTGGTTCTGGGCTGGACTCCAGCCCCTTCCTGTCGGAGGCCAATGCCGAG
CGGATTGTGCAGACCTTATGTACAGTTCGAGGGGCCGCCCTCAAGGTTGGCCAGATGCTC
AGCATCCAGGACAACAGCTTCATCAGCCCTCAGCTGCAGCACATCTTTGAGCGGGTCCGC
CAGAGCGCCGACTTCATGCCCCGCTGGCAGATGCTGAGAGTTCTTGAAGAGGAGCTCGGC
AGGGACTGGCAGGCCAAGGTGGCCTCCTTGGAGGAGGTGCCCTTTGCCGCTGCCTCAATT
GGGCAGGTGCACCAGGGCCTGCTGAGGGACGGGACGGAGGTGGCCGTGAAGATCCAGTAC
CCCGGCATAGCCCAGAGCATTCAGAGCGATGTCCAGAACCTGCTGGCGGTACTCAAGATG
AGCGCGGCCCTGCCCGCGGGCCTGTTTGCCGAGCAGAGCCTGCAGGCCTTGCAGCAGGAG
CTGGCTTGGGAGTGTGACTACCGTCGTGAGGCGGCTTGTGCCCAGAATTTCAGGCAGCTG
CTGGCAAATGACCCCTTCTTCCGGGTCCCAGCCGTGGTTAAGGAGCTGTGCACGACACGG
GTGCTGGGCATGGAGCTGGCTGGAGGGGTCCCCCTGGACCAGTGCCAGGGCCTAAGCCAG
GACCTGCGGAACCAGATTTGCTTCCAGCTCCTGACGCTGTGTCTGCGGGAGCTGTTTGAG
TTCCGATTCATGCAGACTGACCCCAACTGGGCCAACTTCCTGTATGATGCCTCCAGCCAC
CAGGTGACCCTGCTGGACTTTGGTGCAAGCCGGGAGTTTGGGACAGAGTTCACAGACCAT
TACATCGAGGTGGTGAAGGCTGCAGCTGATGGAGACAGAGACTGTGTCCTGCAGAAGTCC
AGGGACCTCAAATTCCTCACAGGCTTTGAAACCAAGGCATTCTCCGACGCCCACGTGGAG
GCAGTGATGATCCTGGGGGAGCCTTTCGCCACCCAGGGCCCTTATGACTTTGGGTCGGGG
GAAACGGCCCGCCGCATACAGGACCTCATCCCGGTGCTGCTGCGGCACCGGCTGTGTCCC
CCACCCGAGGAGACCTATGCCCTGCACCGCAAGCTGGCAGGGGCTTTCCTGGCCTGTGCC
CACCTCCGAGCCCACATCGCCTGCAGGGACCTCTTCCAGGACACCTACCACCGCTACTGG
GCCAGTCGCCAGCCAGACGCAGCCACTGCCGGCAGCCTCCCCACCAAAGGGGACTCCTGG
GTGGATCCCTCATGA
Chromosome Location
19
Locus
19q13.2
External Identifiers
ResourceLink
UniProtKB IDQ96D53
UniProtKB Entry NameCOQ8B_HUMAN
HGNC IDHGNC:19041
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  2. 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 TO, 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: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]
  3. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. [Article]
  4. Khadria AS, Mueller BK, Stefely JA, Tan CH, Pagliarini DJ, Senes A: A Gly-zipper motif mediates homodimerization of the transmembrane domain of the mitochondrial kinase ADCK3. J Am Chem Soc. 2014 Oct 8;136(40):14068-77. doi: 10.1021/ja505017f. Epub 2014 Sep 24. [Article]
  5. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. [Article]
  6. Floyd BJ, Wilkerson EM, Veling MT, Minogue CE, Xia C, Beebe ET, Wrobel RL, Cho H, Kremer LS, Alston CL, Gromek KA, Dolan BK, Ulbrich A, Stefely JA, Bohl SL, Werner KM, Jochem A, Westphall MS, Rensvold JW, Taylor RW, Prokisch H, Kim JP, Coon JJ, Pagliarini DJ: Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function. Mol Cell. 2016 Aug 18;63(4):621-632. doi: 10.1016/j.molcel.2016.06.033. Epub 2016 Aug 4. [Article]
  7. Greenman C, Stephens P, Smith R, Dalgliesh GL, Hunter C, Bignell G, Davies H, Teague J, Butler A, Stevens C, Edkins S, O'Meara S, Vastrik I, Schmidt EE, Avis T, Barthorpe S, Bhamra G, Buck G, Choudhury B, Clements J, Cole J, Dicks E, Forbes S, Gray K, Halliday K, Harrison R, Hills K, Hinton J, Jenkinson A, Jones D, Menzies A, Mironenko T, Perry J, Raine K, Richardson D, Shepherd R, Small A, Tofts C, Varian J, Webb T, West S, Widaa S, Yates A, Cahill DP, Louis DN, Goldstraw P, Nicholson AG, Brasseur F, Looijenga L, Weber BL, Chiew YE, DeFazio A, Greaves MF, Green AR, Campbell P, Birney E, Easton DF, Chenevix-Trench G, Tan MH, Khoo SK, Teh BT, Yuen ST, Leung SY, Wooster R, Futreal PA, Stratton MR: Patterns of somatic mutation in human cancer genomes. Nature. 2007 Mar 8;446(7132):153-8. [Article]
  8. Ashraf S, Gee HY, Woerner S, Xie LX, Vega-Warner V, Lovric S, Fang H, Song X, Cattran DC, Avila-Casado C, Paterson AD, Nitschke P, Bole-Feysot C, Cochat P, Esteve-Rudd J, Haberberger B, Allen SJ, Zhou W, Airik R, Otto EA, Barua M, Al-Hamed MH, Kari JA, Evans J, Bierzynska A, Saleem MA, Bockenhauer D, Kleta R, El Desoky S, Hacihamdioglu DO, Gok F, Washburn J, Wiggins RC, Choi M, Lifton RP, Levy S, Han Z, Salviati L, Prokisch H, Williams DS, Pollak M, Clarke CF, Pei Y, Antignac C, Hildebrandt F: ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption. J Clin Invest. 2013 Dec;123(12):5179-89. doi: 10.1172/JCI69000. Epub 2013 Nov 25. [Article]
  9. Korkmaz E, Lipska-Zietkiewicz BS, Boyer O, Gribouval O, Fourrage C, Tabatabaei M, Schnaidt S, Gucer S, Kaymaz F, Arici M, Dinckan A, Mir S, Bayazit AK, Emre S, Balat A, Rees L, Shroff R, Bergmann C, Mourani C, Antignac C, Ozaltin F, Schaefer F: ADCK4-Associated Glomerulopathy Causes Adolescence-Onset FSGS. J Am Soc Nephrol. 2016 Jan;27(1):63-8. doi: 10.1681/ASN.2014121240. Epub 2015 May 12. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB12010Fostamatinibapproved, investigationalunknowninhibitorDetails