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Identification
HMDB Protein ID HMDBP08626
Secondary Accession Numbers
  • 14341
Name ATP-binding cassette sub-family B member 6, mitochondrial
Synonyms
  1. Mitochondrial ABC transporter 3
  2. Mt-ABC transporter 3
  3. P-glycoprotein-related protein
  4. Ubiquitously-expressed mammalian ABC half transporter
Gene Name ABCB6
Protein Type Unknown
Biological Properties
General Function Involved in ATP binding
Specific Function Binds heme and porphyrins and functions in their ATP- dependent uptake into the mitochondria. Plays a crucial role in heme synthesis
Pathways Not Available
Reactions Not Available
GO Classification
Component
cell part
membrane part
intrinsic to membrane
integral to membrane
Function
active transmembrane transporter activity
primary active transmembrane transporter activity
p-p-bond-hydrolysis-driven transmembrane transporter activity
atpase activity, coupled to transmembrane movement of substances
binding
nucleotide binding
catalytic activity
hydrolase activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
transmembrane transporter activity
atpase activity
transporter activity
nucleoside-triphosphatase activity
hydrolase activity, acting on acid anhydrides
hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides
pyrophosphatase activity
Process
establishment of localization
transport
transmembrane transport
Cellular Location
  1. Multi-pass membrane protein
  2. Mitochondrion outer membrane
Gene Properties
Chromosome Location Chromosome:2
Locus 2q36
SNPs ABCB6
Gene Sequence
>2529 bp
ATGGTGACTGTGGGCAACTACTGCGAGGCCGAAGGGCCCGTGGGTCCGGCCTGGATGCAG
GATGGCCTGAGTCCCTGCTTCTTCTTCACGCTCGTGCCCTCGACGCGGATGGCTCTAGGG
ACTCTGGCCTTGGTGCTGGCTCTTCCCTGCAGACGCCGGGAGCGGCCCGCTGGTGCTGAT
TCGCTGTCTTGGGGGGCCGGCCCTCGCATCTCTCCCTACGTGCTGCAGCTGCTTCTGGCC
ACACTTCAGGCGGCGCTGCCCCTGGCCGGCCTGGCTGGCCGGGTGGGCACTGCCCGGGGG
GCCCCACTGCCAAGCTATCTACTTCTGGCCTCCGTGCTGGAGAGTCTGGCCGGCGCCTGT
GGCCTGTGGCTGCTTGTCGTGGAGCGGAGCCAGGCACGGCAGCGTCTGGCAATGGGCATC
TGGATCAAGTTCAGGCACAGCCCTGGTCTCCTGCTCCTCTGGACTGTGGCGTTTGCAGCT
GAGAACTTGGCCCTGGTGTCTTGGAACAGCCCACAGTGGTGGTGGGCAAGGGCAGACTTG
GGCCAGCAGGTTCAGTTTAGCCTGTGGGTGCTGCGGTATGTGGTCTCTGGAGGGCTGTTT
GTCCTGGGTCTCTGGGCCCCTGGACTTCGTCCCCAGTCCTATACATTGCAGGTTCATGAA
GAGGACCAAGATGTGGAAAGGAGCCAGGTTCGGTCAGCAGCCCAACAGTCTACCTGGCGA
GATTTTGGCAGGAAGCTCCGCCTCCTGAGTGGCTACCTGTGGCCTCGAGGGAGTCCAGCT
CTGCAGCTGGTGGTGCTCATCTGCCTGGGGCTCATGGGTTTGGAACGGGCACTCAATGTG
TTGGTGCCTATATTCTATAGGAACATTGTGAACTTGCTGACTGAGAAGGCACCTTGGAAC
TCTCTGGCCTGGACTGTTACCAGTTACGTCTTCCTCAAGTTCCTCCAGGGGGGTGGCACT
GGCAGTACAGGCTTCGTGAGCAACCTGCGCACCTTCCTGTGGATCCGGGTGCAGCAGTTC
ACGTCTCGGCGGGTGGAGCTGCTCATCTTCTCCCACCTGCACGAGCTCTCACTGCGCTGG
CACCTGGGGCGCCGCACAGGGGAGGTGCTGCGGATCGCGGATCGGGGCACATCCAGTGTC
ACAGGGCTGCTCAGCTACCTGGTGTTCAATGTCATCCCCACGCTGGCCGACATCATCATT
GGCATCATCTACTTCAGCATGTTCTTCAACGCCTGGTTTGGCCTCATTGTGTTCCTGTGC
ATGAGTCTTTACCTCACCCTGACCATTGTGGTCACTGAGTGGAGGACCAAGTTTCGTCGT
GCTATGAACACACAGGAGAACGCTACCCGGGCACGAGCAGTGGACTCTCTGCTAAACTTC
GAGACGGTGAAGTATTACAACGCCGAGAGTTACGAAGTGGAACGCTATCGAGAGGCCATC
ATCAAATATCAGGGTTTGGAGTGGAAGTCGAGCGCTTCACTGGTTTTACTAAATCAGACC
CAGAACCTGGTGATTGGGCTCGGGCTCCTCGCCGGCTCCCTGCTTTGCGCATACTTTGTC
ACTGAGCAGAAGCTACAGGTTGGGGACTATGTGCTCTTTGGCACCTACATTATCCAGCTG
TACATGCCCCTCAATTGGTTTGGCACCTACTACAGGATGATCCAGACCAACTTCATTGAC
ATGGAGAACATGTTTGACTTGCTGAAAGAGGAGACAGAAGTGAAGGACCTTCCTGGAGCA
GGGCCCCTTCGCTTTCAGAAGGGCCGTATTGAGTTTGAGAACGTGCACTTCAGCTATGCC
GATGGGCGGGAGACTCTGCAGGACGTGTCTTTCACTGTGATGCCTGGACAGACACTTGCC
CTGGTGGGCCCATCTGGGGCAGGGAAGAGCACAATTTTGCGCCTGCTGTTTCGCTTCTAC
GACATCAGCTCTGGCTGCATCCGAATAGATGGGCAGGACATTTCACAGGTGACCCAGGCC
TCTCTCCGGTCTCACATTGGAGTTGTGCCCCAAGACACTGTCCTCTTTAATGACACCATC
GCCGACAATATCCGTTACGGCCGTGTCACAGCTGGGAATGATGAGGTGGAGGCTGCTGCT
CAGGCTGCAGGCATCCATGATGCCATTATGGCTTTCCCTGAAGGGTACAGGACACAGGTG
GGCGAGCGGGGACTGAAGCTGAGCGGCGGGGAGAAGCAGCGCGTCGCCATTGCCCGCACC
ATCCTCAAGGCTCCGGGCATCATTCTGCTGGATGAGGCAACGTCAGCGCTGGATACATCT
AATGAGAGGGCCATCCAGGCTTCTCTGGCCAAAGTCTGTGCCAACCGCACCACCATCGTA
GTGGCACACAGGCTCTCAACTGTGGTCAATGCTGACCAGATCCTCGTCATCAAGGATGGC
TGCATCGTGGAGAGGGGACGACACGAGGCTCTGTTGTCCCGAGGTGGGGTGTATGCTGAC
ATGTGGCAGCTGCAGCAGGGACAGGAAGAAACCTCTGAAGACACTAAGCCTCAGACCATG
GAACGGTGA
Protein Properties
Number of Residues 842
Molecular Weight 93884.7
Theoretical pI 8.61
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • 27-47
  • 73-93
  • 107-127
  • 148-168
  • 186-206
  • 265-285
  • 376-396
  • 409-431
  • 502-522
  • 530-550
Protein Sequence
>ATP-binding cassette sub-family B member 6, mitochondrial
MVTVGNYCEAEGPVGPAWMQDGLSPCFFFTLVPSTRMALGTLALVLALPCRRRERPAGAD
SLSWGAGPRISPYVLQLLLATLQAALPLAGLAGRVGTARGAPLPSYLLLASVLESLAGAC
GLWLLVVERSQARQRLAMGIWIKFRHSPGLLLLWTVAFAAENLALVSWNSPQWWWARADL
GQQVQFSLWVLRYVVSGGLFVLGLWAPGLRPQSYTLQVHEEDQDVERSQVRSAAQQSTWR
DFGRKLRLLSGYLWPRGSPALQLVVLICLGLMGLERALNVLVPIFYRNIVNLLTEKAPWN
SLAWTVTSYVFLKFLQGGGTGSTGFVSNLRTFLWIRVQQFTSRRVELLIFSHLHELSLRW
HLGRRTGEVLRIADRGTSSVTGLLSYLVFNVIPTLADIIIGIIYFSMFFNAWFGLIVFLC
MSLYLTLTIVVTEWRTKFRRAMNTQENATRARAVDSLLNFETVKYYNAESYEVERYREAI
IKYQGLEWKSSASLVLLNQTQNLVIGLGLLAGSLLCAYFVTEQKLQVGDYVLFGTYIIQL
YMPLNWFGTYYRMIQTNFIDMENMFDLLKEETEVKDLPGAGPLRFQKGRIEFENVHFSYA
DGRETLQDVSFTVMPGQTLALVGPSGAGKSTILRLLFRFYDISSGCIRIDGQDISQVTQA
SLRSHIGVVPQDTVLFNDTIADNIRYGRVTAGNDEVEAAAQAAGIHDAIMAFPEGYRTQV
GERGLKLSGGEKQRVAIARTILKAPGIILLDEATSALDTSNERAIQASLAKVCANRTTIV
VAHRLSTVVNADQILVIKDGCIVERGRHEALLSRGGVYADMWQLQQGQEETSEDTKPQTM
ER
GenBank ID Protein 8547018
UniProtKB/Swiss-Prot ID Q9NP58
UniProtKB/Swiss-Prot Entry Name ABCB6_HUMAN
PDB IDs Not Available
GenBank Gene ID AB039371
GeneCard ID ABCB6
GenAtlas ID ABCB6
HGNC ID HGNC:47
References
General References
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  2. 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. [PubMed:15489334 ]
  3. 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. [PubMed:16959974 ]
  4. Mitsuhashi N, Miki T, Senbongi H, Yokoi N, Yano H, Miyazaki M, Nakajima N, Iwanaga T, Yokoyama Y, Shibata T, Seino S: MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis. J Biol Chem. 2000 Jun 9;275(23):17536-40. [PubMed:10837493 ]
  5. Emadi-Konjin HP, Zhang H, Anandan V, Sun D, Schuetz J, Furuya KN: Isolation of a genomic clone containing the promoter region of the human ATP binding cassette (ABC) transporter, ABCB6. Biochim Biophys Acta. 2002 Mar 19;1574(2):117-30. [PubMed:11955620 ]
  6. Krishnamurthy PC, Du G, Fukuda Y, Sun D, Sampath J, Mercer KE, Wang J, Sosa-Pineda B, Murti KG, Schuetz JD: Identification of a mammalian mitochondrial porphyrin transporter. Nature. 2006 Oct 5;443(7111):586-9. Epub 2006 Sep 27. [PubMed:17006453 ]
  7. Kurashima-Ito K, Ikeya T, Senbongi H, Tochio H, Mikawa T, Shibata T, Ito Y: Heteronuclear multidimensional NMR and homology modelling studies of the C-terminal nucleotide-binding domain of the human mitochondrial ABC transporter ABCB6. J Biomol NMR. 2006 May;35(1):53-71. Epub 2006 Jun 8. [PubMed:16791740 ]