Mitochondrial potassium channel ATP-binding subunit

Details

Name
Mitochondrial potassium channel ATP-binding subunit
Kind
protein
Synonyms
  • ABCB8
  • ATP-binding cassette sub-family B member 8, mitochondrial
  • M-ABC1
  • MABC1
  • Mitochondrial ATP-binding cassette 1
  • Mitochondrial sulfonylurea-receptor
  • MITOSUR
Gene Name
ABCB8
UniProtKB Entry
Q9NUT2Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0007059|Mitochondrial potassium channel ATP-binding subunit
MLVHLFRVGIRGGPFPGRLLPPLRFQTFSAVRNTWRNGKTGQLHKAEGEYSDGYRSSSLL
RAVAHLRSQLWAHLPRAPLAPRWSPSAWCWVGGALLGPMVLSKHPHLCLVALCEAEEAPP
ASSTPHVVGSRFNWKLFWQFLHPHLLVLGVAVVLALGAALVNVQIPLLLGQLVEVVAKYT
RDHVGSFMTESQNLSTHLLILYGVQGLLTFGYLVLLSHVGERMAVDMRRALFSSLLRQDI
TFFDANKTGQLVSRLTTDVQEFKSSFKLVISQGLRSCTQVAGCLVSLSMLSTRLTLLLMV
ATPALMGVGTLMGSGLRKLSRQCQEQIARAMGVADEALGNVRTVRAFAMEQREEERYGAE
LEACRCRAEELGRGIALFQGLSNIAFNCMVLGTLFIGGSLVAGQQLTGGDLMSFLVASQT
VQRSMANLSVLFGQVVRGLSAGARVFEYMALNPCIPLSGGCCVPKEQLRGSVTFQNVCFS
YPCRPGFEVLKDFTLTLPPGKIVALVGQSGGGKTTVASLLERFYDPTAGVVMLDGRDLRT
LDPSWLRGQVVGFISQEPVLFGTTIMENIRFGKLEASDEEVYTAAREANAHEFITSFPEG
YNTVVGERGTTLSGGQKQRLAIARALIKQPTVLILDEATSALDAESERVVQEALDRASAG
RTVLVIAHRLSTVRGAHCIVVMADGRVWEAGTHEELLKKGGLYAELIRRQALDAPRTAAP
PPKKPEGPRSHQHKS
Number of residues
735
Molecular Weight
79988.17
Theoretical pI
9.18
GO Classification
Functions
ATP binding
Processes
transmembrane transport
Components
ATP-binding cassette (ABC) transporter complex / membrane / mitochondrial inner membrane / mitochondrion / nucleolus
General Function
ATP-binding subunit of the mitochondrial ATP-gated potassium channel (mitoK(ATP)) (PubMed:31435016). Together with pore-forming subunit CCDC51/MITOK of the mitoK(ATP) channel, mediates ATP-dependent potassium currents across the mitochondrial inner membrane (PubMed:31435016). An increase in ATP intracellular levels closes the channel, inhibiting K(+) transport, whereas a decrease in ATP levels enhances K(+) uptake in the mitochondrial matrix (PubMed:31435016). Plays a role in mitochondrial iron transport (PubMed:30623799). Required for maintenance of normal cardiac function, possibly by influencing mitochondrial iron export and regulating the maturation of cytosolic iron sulfur cluster-containing enzymes (By similarity)
Specific Function
Abc-type peptide transporter activity
Pfam Domain Function
Signal Regions
Not Available
Transmembrane Regions
145-165 196-216 296-316
Cellular Location
Mitochondrion inner membrane
Gene sequence
>lcl|BSEQ0012552|ATP-binding cassette sub-family B member 8, mitochondrial (ABCB8)
ATGCTGGTGCATTTATTTCGGGTCGGGATTCGGGGTGGCCCATTCCCAGGCAGGCTGCTA
CCGCCCCTCCGCTTCCAGACATTCTCAGCTGTCAGAAACACATGGCGTAATGGGAAAACT
GGACAGTTACACAAGGCAGAAGGAGAGTACTCTGATGGCTACCGCAGCTCCTCCCTCCTC
CGGGCCGTGGCCCACCTGCGGTCCCAGCTCTGGGCCCACCTCCCTCGAGCCCCCCTAGCT
CCCAGATGGAGCCCCTCTGCCTGGTGCTGGGTTGGGGGAGCCCTGCTAGGCCCCATGGTA
CTGAGTAAGCATCCCCACCTCTGCCTTGTGGCCCTGTGTGAGGCAGAAGAGGCCCCTCCT
GCCAGCTCCACACCCCATGTCGTGGGGTCTCGCTTTAACTGGAAGCTCTTCTGGCAGTTT
CTGCACCCCCACCTGCTGGTCCTGGGGGTAGCCGTCGTGCTGGCCTTGGGTGCGGCACTC
GTGAATGTACAGATCCCCCTGCTCCTGGGCCAGCTGGTAGAGGTCGTGGCCAAGTACACA
AGGGACCACGTAGGGAGTTTCATGACTGAGTCCCAGAATCTCAGCACCCACCTGCTTATC
CTCTATGGTGTCCAGGGACTGCTGACCTTCGGGTACCTGGTGCTGCTGTCCCACGTTGGC
GAGCGCATGGCTGTGGACATGCGGAGGGCCCTCTTCAGCTCCCTGCTCCGACAAGACATC
ACCTTCTTTGACGCCAATAAGACAGGGCAGCTGGTGAGCCGCTTGACAACTGACGTGCAG
GAGTTTAAGTCATCCTTCAAGCTTGTCATCTCCCAGGGGCTGCGAAGCTGCACCCAGGTG
GCAGGCTGCCTGGTGTCCCTGTCCATGCTGTCGACACGCCTCACGCTGCTGCTGATGGTG
GCCACACCAGCCCTGATGGGAGTGGGCACCCTGATGGGCTCAGGCCTCCGAAAATTGTCT
CGCCAGTGTCAGGAGCAGATCGCCAGGGCAATGGGCGTAGCAGACGAGGCCCTGGGCAAT
GTGCGGACTGTGCGTGCCTTCGCCATGGAGCAACGGGAAGAGGAGCGCTATGGGGCAGAG
CTGGAAGCCTGCCGCTGCCGGGCAGAGGAGCTGGGCCGCGGCATCGCCTTGTTCCAAGGG
CTTTCCAACATCGCCTTCAACTGCATGGTCTTGGGTACCCTATTTATTGGGGGCTCCCTT
GTGGCCGGACAGCAGCTGACAGGGGGAGACCTCATGTCCTTCCTGGTGGCCTCCCAGACA
GTGCAAAGGTCCATGGCCAACCTCTCTGTCCTGTTTGGGCAGGTGGTCCGGGGGCTGAGT
GCAGGTGCCCGGGTCTTTGAGTACATGGCCCTGAACCCCTGCATCCCACTGTCTGGGGGC
TGCTGCGTCCCCAAAGAGCAGCTGCGTGGCTCCGTTACATTTCAGAACGTCTGCTTCAGC
TACCCCTGCCGCCCCGGCTTCGAGGTGCTGAAAGACTTCACCCTGACGCTGCCCCCTGGC
AAGATCGTGGCCCTCGTGGGCCAGTCTGGCGGAGGAAAGACCACCGTGGCTTCCCTGCTG
GAGCGCTTCTACGACCCCACGGCAGGCGTGGTGATGCTGGATGGGCGGGACCTGCGCACC
CTTGACCCCTCCTGGCTCCGGGGCCAGGTTGTCGGCTTCATCAGCCAGGAGCCCGTCCTG
TTTGGGACGACCATCATGGAAAACATCCGCTTTGGGAAGCTGGAAGCTTCCGATGAAGAG
GTGTACACAGCCGCCCGGGAAGCGAATGCTCACGAGTTCATCACCAGCTTCCCCGAGGGC
TACAACACGGTCGTCGGTGAACGGGGCACTACCCTGTCTGGGGGCCAGAAGCAGCGCCTG
GCCATCGCCCGAGCCCTTATCAAGCAGCCCACGGTGCTGATACTGGATGAAGCTACCAGC
GCGCTGGATGCAGAGTCCGAGCGGGTTGTACAGGAGGCCCTGGACCGGGCCAGTGCAGGC
CGCACGGTGCTGGTAATTGCCCACCGGCTCAGCACTGTCCGTGGGGCCCACTGCATTGTC
GTCATGGCCGATGGCCGTGTCTGGGAGGCTGGGACACATGAAGAGCTCCTGAAGAAAGGC
GGGCTATACGCCGAGCTCATCCGGAGGCAGGCCCTGGATGCCCCGAGGACAGCGGCCCCA
CCGCCCAAAAAGCCAGAAGGCCCCAGGAGCCACCAGCACAAGTCCTGA
Chromosome Location
7
Locus
7q36.1
External Identifiers
ResourceLink
UniProtKB IDQ9NUT2
UniProtKB Entry NameMITOS_HUMAN
GenBank Protein ID4321407
GenBank Gene IDAF047690
GeneCard IDABCB8
HGNC IDHGNC:49
PDB ID(s)5OCH, 7EHL
KEGG IDhsa:11194
NCBI Gene ID11194
General References
  1. Hogue DL, Liu L, Ling V: Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein. J Mol Biol. 1999 Jan 8;285(1):379-89. [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]
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  4. 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]
  5. Ardehali H, Xue T, Dong P, Machamer C: Targeting of the mitochondrial membrane proteins to the cell surface for functional studies. Biochem Biophys Res Commun. 2005 Dec 16;338(2):1143-51. Epub 2005 Oct 21. [Article]
  6. 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]
  7. Saito S, Iida A, Sekine A, Miura Y, Ogawa C, Kawauchi S, Higuchi S, Nakamura Y: Three hundred twenty-six genetic variations in genes encoding nine members of ATP-binding cassette, subfamily B (ABCB/MDR/TAP), in the Japanese population. J Hum Genet. 2002;47(1):38-50. [Article]
  8. Sjoblom T, Jones S, Wood LD, Parsons DW, Lin J, Barber TD, Mandelker D, Leary RJ, Ptak J, Silliman N, Szabo S, Buckhaults P, Farrell C, Meeh P, Markowitz SD, Willis J, Dawson D, Willson JK, Gazdar AF, Hartigan J, Wu L, Liu C, Parmigiani G, Park BH, Bachman KE, Papadopoulos N, Vogelstein B, Kinzler KW, Velculescu VE: The consensus coding sequences of human breast and colorectal cancers. Science. 2006 Oct 13;314(5797):268-74. Epub 2006 Sep 7. [Article]

Associated Data

Drug Relations
DrugDrug groupPharmacological action?TypeActionsDetails
Doxorubicinapproved, investigationalunknowntransportersubstrateDetails