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Q9NRK6 (ABCBA_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified March 19, 2014. Version 124. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (6) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
ATP-binding cassette sub-family B member 10, mitochondrial
Alternative name(s):
ATP-binding cassette transporter 10
Short name=ABC transporter 10 protein
Mitochondrial ATP-binding cassette 2
Short name=M-ABC2
Gene names
Name:ABCB10
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length738 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

May mediate critical mitochondrial transport functions related to heme biosynthesis By similarity.

Subunit structure

Homodimer or homooligomer. Ref.7 Ref.10

Subcellular location

Mitochondrion inner membrane; Multi-pass membrane protein.

Tissue specificity

Ubiquitous. Highly expressed in bone marrow, expressed at intermediate to high levels in skeletal muscle, small intestine, thyroid, heart, brain, placenta, liver, pancreas, prostate, testis, ovary, leukocyte, stomach, spinal cord, lymph node, trachea and adrenal gland, and low levels are found in lung, kidney, spleen, thymus and colon.

Sequence similarities

Belongs to the ABC transporter superfamily. ABCB family. Mitochondrial peptide exporter (TC 3.A.1.212) subfamily. [View classification]

Contains 1 ABC transmembrane type-1 domain.

Contains 1 ABC transporter domain.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 105105Mitochondrion
Chain106 – 738633ATP-binding cassette sub-family B member 10, mitochondrial
PRO_0000000255

Regions

Topological domain? – 170Mitochondrial intermembrane Potential
Transmembrane171 – 19121Helical; Potential
Topological domain192 – 21524Mitochondrial matrix Potential
Transmembrane216 – 23621Helical; Potential
Topological domain237 – 31276Mitochondrial intermembrane Potential
Transmembrane313 – 33321Helical; Potential
Topological domain334 – 40774Mitochondrial matrix Potential
Transmembrane408 – 42821Helical; Potential
Topological domain429 – 4302Mitochondrial intermembrane Potential
Transmembrane431 – 45121Helical; Potential
Topological domain452 – 738287Mitochondrial matrix Potential
Domain171 – 457287ABC transmembrane type-1
Domain492 – 731240ABC transporter
Nucleotide binding527 – 5348ATP

Amino acid modifications

Modified residue2651N6-acetyllysine Ref.8

Natural variations

Natural variant1501A → S. Ref.11
Corresponds to variant rs4148756 [ dbSNP | Ensembl ].
VAR_013702
Natural variant2421R → G.
Corresponds to variant rs17584642 [ dbSNP | Ensembl ].
VAR_048133
Natural variant4711R → T in a breast cancer sample; somatic mutation. Ref.12
VAR_035735
Natural variant5451D → N. Ref.1
Corresponds to variant rs35698797 [ dbSNP | Ensembl ].
VAR_031435

Experimental info

Sequence conflict3931F → V in AAF78198. Ref.1
Sequence conflict5111Q → K in AAA84438. Ref.6
Sequence conflict558 – 5636IRQLNP → NPSAKPS in AAA84438. Ref.6
Sequence conflict606 – 6116VAEVAN → GLKGQ in BAB20265. Ref.2
Sequence conflict615 – 6228FIRNFPQG → SPEFPPR in AAA84438. Ref.6
Sequence conflict6911R → S in AAA84438. Ref.6

Secondary structure

........................................................................ 738
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
Q9NRK6 [UniParc].

Last modified April 3, 2007. Version 2.
Checksum: C68B4FAC0F8B7E43

FASTA73879,148
        10         20         30         40         50         60 
MRGPPAWPLR LLEPPSPAEP GRLLPVACVW AAASRVPGSL SPFTGLRPAR LWGAGPALLW 

        70         80         90        100        110        120 
GVGAARRWRS GCRGGGPGAS RGVLGLARLL GLWARGPGSC RCGAFAGPGA PRLPRARFPG 

       130        140        150        160        170        180 
GPAAAAWAGD EAWRRGPAAP PGDKGRLRPA AAGLPEARKL LGLAYPERRR LAAAVGFLTM 

       190        200        210        220        230        240 
SSVISMSAPF FLGKIIDVIY TNPTVDYSDN LTRLCLGLSA VFLCGAAANA IRVYLMQTSG 

       250        260        270        280        290        300 
QRIVNRLRTS LFSSILRQEV AFFDKTRTGE LINRLSSDTA LLGRSVTENL SDGLRAGAQA 

       310        320        330        340        350        360 
SVGISMMFFV SPNLATFVLS VVPPVSIIAV IYGRYLRKLT KVTQDSLAQA TQLAEERIGN 

       370        380        390        400        410        420 
VRTVRAFGKE MTEIEKYASK VDHVMQLARK EAFARAGFFG ATGLSGNLIV LSVLYKGGLL 

       430        440        450        460        470        480 
MGSAHMTVGE LSSFLMYAFW VGISIGGLSS FYSELMKGLG AGGRLWELLE REPKLPFNEG 

       490        500        510        520        530        540 
VILNEKSFQG ALEFKNVHFA YPARPEVPIF QDFSLSIPSG SVTALVGPSG SGKSTVLSLL 

       550        560        570        580        590        600 
LRLYDPASGT ISLDGHDIRQ LNPVWLRSKI GTVSQEPILF SCSIAENIAY GADDPSSVTA 

       610        620        630        640        650        660 
EEIQRVAEVA NAVAFIRNFP QGFNTVVGEK GVLLSGGQKQ RIAIARALLK NPKILLLDEA 

       670        680        690        700        710        720 
TSALDAENEY LVQEALDRLM DGRTVLVIAH RLSTIKNANM VAVLDQGKIT EYGKHEELLS 

       730 
KPNGIYRKLM NKQSFISA 

« Hide

References

« Hide 'large scale' references
[1]"M-ABC2, a new human mitochondrial ATP-binding cassette membrane protein."
Zhang F., Hogue D.L., Liu L., Fisher C.L., Hui D., Childs S., Ling V.
FEBS Lett. 478:89-94(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT ASN-545.
Tissue: Lymphoblast.
[2]"Human mono ATP-binding cassette protein."
Ito K., Suzuki H., Sugiyama Y.
Submitted (APR-1998) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[3]"The DNA sequence and biological annotation of human chromosome 1."
Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K. expand/collapse author list , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R.
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Eye.
[6]"Characterization and mapping of three new mammalian ATP-binding transporter genes from an EST database."
Allikmets R., Gerrard B., Glavac D., Ravnik-Glavac M., Jenkins N.A., Gilbert D.J., Copeland N.G., Modi W., Dean M.
Mamm. Genome 6:114-117(1995) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 510-738.
[7]"Targeting, import, and dimerization of a mammalian mitochondrial ATP binding cassette (ABC) transporter, ABCB10 (ABC-me)."
Graf S.A., Haigh S.E., Corson E.D., Shirihai O.S.
J. Biol. Chem. 279:42954-42963(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: TRANSIT PEPTIDE, SUBUNIT.
[8]"Lysine acetylation targets protein complexes and co-regulates major cellular functions."
Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-265, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[9]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[10]"Structures of ABCB10, a human ATP-binding cassette transporter in apo- and nucleotide-bound states."
Shintre C.A., Pike A.C., Li Q., Kim J.I., Barr A.J., Goubin S., Shrestha L., Yang J., Berridge G., Ross J., Stansfeld P.J., Sansom M.S., Edwards A.M., Bountra C., Marsden B.D., von Delft F., Bullock A.N., Gileadi O., Burgess-Brown N.A., Carpenter E.P.
Proc. Natl. Acad. Sci. U.S.A. 110:9710-9715(2013) [PubMed] [Europe PMC] [Abstract]
Cited for: X-RAY CRYSTALLOGRAPHY (2.85 ANGSTROMS) OF 152-738 ALONE AND IN COMPLEX WITH ATP ANALOGS, ATP-BINDING REGION, SUBUNIT.
[11]"Three hundred twenty-six genetic variations in genes encoding nine members of ATP-binding cassette, subfamily B (ABCB/MDR/TAP), in the Japanese population."
Saito S., Iida A., Sekine A., Miura Y., Ogawa C., Kawauchi S., Higuchi S., Nakamura Y.
J. Hum. Genet. 47:38-50(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT SER-150.
[12]"The consensus coding sequences of human breast and colorectal cancers."
Sjoeblom T., Jones S., Wood L.D., Parsons D.W., Lin J., Barber T.D., Mandelker D., Leary R.J., Ptak J., Silliman N., Szabo S., Buckhaults P., Farrell C., Meeh P., Markowitz S.D., Willis J., Dawson D., Willson J.K.V. expand/collapse author list , Gazdar A.F., Hartigan J., Wu L., Liu C., Parmigiani G., Park B.H., Bachman K.E., Papadopoulos N., Vogelstein B., Kinzler K.W., Velculescu V.E.
Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT [LARGE SCALE ANALYSIS] THR-471.
+Additional computationally mapped references.

Web resources

ABCMdb

Database for mutations in ABC proteins

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF216833 mRNA. Translation: AAF78198.1.
AB013380 mRNA. Translation: BAB20265.1.
AL121990 Genomic DNA. Translation: CAI22012.1.
CH471098 Genomic DNA. Translation: EAW69901.1.
BC064930 mRNA. Translation: AAH64930.1.
U18237 mRNA. Translation: AAA84438.1.
RefSeqNP_036221.2. NM_012089.2.
UniGeneHs.17614.

3D structure databases

PDBe
RCSB PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
3ZDQX-ray2.85A152-738[»]
4AYTX-ray2.85A152-738[»]
4AYWX-ray3.30A126-738[»]
4AYXX-ray2.90A152-738[»]
ProteinModelPortalQ9NRK6.
SMRQ9NRK6. Positions 153-722.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid117020. 9 interactions.
IntActQ9NRK6. 1 interaction.
STRING9606.ENSP00000355637.

Protein family/group databases

TCDB3.A.1.201.17. the atp-binding cassette (abc) superfamily.

PTM databases

PhosphoSiteQ9NRK6.

Polymorphism databases

DMDM143811359.

Proteomic databases

PaxDbQ9NRK6.
PRIDEQ9NRK6.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000344517; ENSP00000355637; ENSG00000135776.
GeneID23456.
KEGGhsa:23456.
UCSCuc001htp.4. human.

Organism-specific databases

CTD23456.
GeneCardsGC01M229652.
H-InvDBHIX0028493.
HGNCHGNC:41. ABCB10.
HPACAB044063.
CAB044065.
MIM605454. gene.
neXtProtNX_Q9NRK6.
PharmGKBPA24385.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG1132.
HOVERGENHBG008358.
InParanoidQ9NRK6.
KOK05657.
OMAGTFVRFY.
OrthoDBEOG7Z3F4H.
PhylomeDBQ9NRK6.
TreeFamTF105198.

Enzyme and pathway databases

BRENDA3.6.3.43. 2681.
ReactomeREACT_15518. Transmembrane transport of small molecules.

Gene expression databases

ArrayExpressQ9NRK6.
BgeeQ9NRK6.
CleanExHS_ABCB10.
GenevestigatorQ9NRK6.

Family and domain databases

InterProIPR003593. AAA+_ATPase.
IPR011527. ABC1_TM_dom.
IPR003439. ABC_transporter-like.
IPR017871. ABC_transporter_CS.
IPR001140. ABC_transptr_TM_dom.
IPR027417. P-loop_NTPase.
[Graphical view]
PfamPF00664. ABC_membrane. 1 hit.
PF00005. ABC_tran. 1 hit.
[Graphical view]
SMARTSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMSSF52540. SSF52540. 1 hit.
SSF90123. SSF90123. 1 hit.
PROSITEPS50929. ABC_TM1F. 1 hit.
PS00211. ABC_TRANSPORTER_1. 1 hit.
PS50893. ABC_TRANSPORTER_2. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GenomeRNAi23456.
NextBio45747.
PROQ9NRK6.
SOURCESearch...

Entry information

Entry nameABCBA_HUMAN
AccessionPrimary (citable) accession number: Q9NRK6
Secondary accession number(s): Q13040, Q6P1Q8, Q9H3V0
Entry history
Integrated into UniProtKB/Swiss-Prot: August 2, 2002
Last sequence update: April 3, 2007
Last modified: March 19, 2014
This is version 124 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 1

Human chromosome 1: entries, gene names and cross-references to MIM