Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

ATP-binding cassette sub-family B member 10, mitochondrial

Gene

ABCB10

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi527 – 534ATP8

GO - Molecular functioni

GO - Biological processi

  • transmembrane transport Source: GO_Central
  • transport Source: UniProtKB
Complete GO annotation...

Keywords - Biological processi

Transport

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000135776-MONOMER.
BRENDAi3.6.3.43. 2681.
ReactomeiR-HSA-1369007. Mitochondrial ABC transporters.

Protein family/group databases

TCDBi3.A.1.201.17. the atp-binding cassette (abc) superfamily.

Names & Taxonomyi

Protein namesi
Recommended 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 namesi
Name:ABCB10
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:41. ABCB10.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini106 – 170Mitochondrial intermembraneSequence analysisAdd BLAST65
Transmembranei171 – 191HelicalPROSITE-ProRule annotationAdd BLAST21
Topological domaini192 – 215Mitochondrial matrixSequence analysisAdd BLAST24
Transmembranei216 – 236HelicalPROSITE-ProRule annotationAdd BLAST21
Topological domaini237 – 312Mitochondrial intermembraneSequence analysisAdd BLAST76
Transmembranei313 – 333HelicalPROSITE-ProRule annotationAdd BLAST21
Topological domaini334 – 407Mitochondrial matrixSequence analysisAdd BLAST74
Transmembranei408 – 428HelicalPROSITE-ProRule annotationAdd BLAST21
Topological domaini429 – 430Mitochondrial intermembraneSequence analysis2
Transmembranei431 – 451HelicalPROSITE-ProRule annotationAdd BLAST21
Topological domaini452 – 738Mitochondrial matrixSequence analysisAdd BLAST287

GO - Cellular componenti

  • integral component of mitochondrial membrane Source: UniProtKB
  • mitochondrial inner membrane Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi23456.
OpenTargetsiENSG00000135776.
PharmGKBiPA24385.

Polymorphism and mutation databases

BioMutaiABCB10.
DMDMi143811359.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 105MitochondrionBy similarityAdd BLAST105
ChainiPRO_0000000255106 – 738ATP-binding cassette sub-family B member 10, mitochondrialAdd BLAST633

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei265N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9NRK6.
MaxQBiQ9NRK6.
PaxDbiQ9NRK6.
PeptideAtlasiQ9NRK6.
PRIDEiQ9NRK6.

PTM databases

iPTMnetiQ9NRK6.
PhosphoSitePlusiQ9NRK6.
SwissPalmiQ9NRK6.

Expressioni

Tissue specificityi

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.

Gene expression databases

BgeeiENSG00000135776.
CleanExiHS_ABCB10.
GenevisibleiQ9NRK6. HS.

Organism-specific databases

HPAiCAB044063.
CAB044065.
HPA055175.

Interactioni

Subunit structurei

Homodimer or homooligomer.1 Publication

Protein-protein interaction databases

BioGridi117020. 25 interactors.
IntActiQ9NRK6. 2 interactors.
STRINGi9606.ENSP00000355637.

Structurei

Secondary structure

1738
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi154 – 164Combined sources11
Helixi165 – 167Combined sources3
Helixi168 – 185Combined sources18
Helixi188 – 201Combined sources14
Helixi208 – 255Combined sources48
Helixi260 – 265Combined sources6
Helixi268 – 285Combined sources18
Helixi288 – 310Combined sources23
Helixi312 – 359Combined sources48
Helixi361 – 366Combined sources6
Helixi370 – 422Combined sources53
Helixi428 – 469Combined sources42
Turni485 – 487Combined sources3
Beta strandi492 – 499Combined sources8
Beta strandi502 – 504Combined sources3
Beta strandi509 – 517Combined sources9
Beta strandi522 – 526Combined sources5
Helixi530 – 540Combined sources11
Beta strandi547 – 553Combined sources7
Helixi558 – 560Combined sources3
Helixi563 – 568Combined sources6
Beta strandi569 – 573Combined sources5
Beta strandi581 – 583Combined sources3
Helixi584 – 589Combined sources6
Beta strandi592 – 594Combined sources3
Turni595 – 597Combined sources3
Helixi600 – 609Combined sources10
Helixi613 – 617Combined sources5
Beta strandi619 – 621Combined sources3
Helixi622 – 624Combined sources3
Beta strandi628 – 631Combined sources4
Helixi636 – 650Combined sources15
Beta strandi653 – 658Combined sources6
Helixi666 – 680Combined sources15
Beta strandi683 – 688Combined sources6
Helixi692 – 697Combined sources6
Beta strandi698 – 704Combined sources7
Beta strandi706 – 713Combined sources8
Helixi715 – 719Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3ZDQX-ray2.85A152-738[»]
4AYTX-ray2.85A152-738[»]
4AYWX-ray3.30A1-738[»]
4AYXX-ray2.90A152-738[»]
ProteinModelPortaliQ9NRK6.
SMRiQ9NRK6.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini171 – 457ABC transmembrane type-1PROSITE-ProRule annotationAdd BLAST287
Domaini492 – 731ABC transporterPROSITE-ProRule annotationAdd BLAST240

Sequence similaritiesi

Contains 1 ABC transmembrane type-1 domain.PROSITE-ProRule annotation
Contains 1 ABC transporter domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0058. Eukaryota.
COG1132. LUCA.
GeneTreeiENSGT00550000074497.
HOVERGENiHBG008358.
InParanoidiQ9NRK6.
KOiK05657.
OMAiWAGDEAW.
OrthoDBiEOG091G0HVA.
PhylomeDBiQ9NRK6.
TreeFamiTF105198.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR011527. ABC1_TM_dom.
IPR003439. ABC_transporter-like.
IPR017871. ABC_transporter_CS.
IPR030260. ABCB10.
IPR027417. P-loop_NTPase.
[Graphical view]
PANTHERiPTHR24221:SF121. PTHR24221:SF121. 1 hit.
PfamiPF00664. ABC_membrane. 1 hit.
PF00005. ABC_tran. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
SSF90123. SSF90123. 1 hit.
PROSITEiPS50929. ABC_TM1F. 1 hit.
PS00211. ABC_TRANSPORTER_1. 1 hit.
PS50893. ABC_TRANSPORTER_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q9NRK6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MRGPPAWPLR LLEPPSPAEP GRLLPVACVW AAASRVPGSL SPFTGLRPAR
60 70 80 90 100
LWGAGPALLW GVGAARRWRS GCRGGGPGAS RGVLGLARLL GLWARGPGSC
110 120 130 140 150
RCGAFAGPGA PRLPRARFPG GPAAAAWAGD EAWRRGPAAP PGDKGRLRPA
160 170 180 190 200
AAGLPEARKL LGLAYPERRR LAAAVGFLTM SSVISMSAPF FLGKIIDVIY
210 220 230 240 250
TNPTVDYSDN LTRLCLGLSA VFLCGAAANA IRVYLMQTSG QRIVNRLRTS
260 270 280 290 300
LFSSILRQEV AFFDKTRTGE LINRLSSDTA LLGRSVTENL SDGLRAGAQA
310 320 330 340 350
SVGISMMFFV SPNLATFVLS VVPPVSIIAV IYGRYLRKLT KVTQDSLAQA
360 370 380 390 400
TQLAEERIGN VRTVRAFGKE MTEIEKYASK VDHVMQLARK EAFARAGFFG
410 420 430 440 450
ATGLSGNLIV LSVLYKGGLL MGSAHMTVGE LSSFLMYAFW VGISIGGLSS
460 470 480 490 500
FYSELMKGLG AGGRLWELLE REPKLPFNEG VILNEKSFQG ALEFKNVHFA
510 520 530 540 550
YPARPEVPIF QDFSLSIPSG SVTALVGPSG SGKSTVLSLL LRLYDPASGT
560 570 580 590 600
ISLDGHDIRQ LNPVWLRSKI GTVSQEPILF SCSIAENIAY GADDPSSVTA
610 620 630 640 650
EEIQRVAEVA NAVAFIRNFP QGFNTVVGEK GVLLSGGQKQ RIAIARALLK
660 670 680 690 700
NPKILLLDEA TSALDAENEY LVQEALDRLM DGRTVLVIAH RLSTIKNANM
710 720 730
VAVLDQGKIT EYGKHEELLS KPNGIYRKLM NKQSFISA
Length:738
Mass (Da):79,148
Last modified:April 3, 2007 - v2
Checksum:iC68B4FAC0F8B7E43
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti393F → V in AAF78198 (PubMed:10922475).Curated1
Sequence conflicti511Q → K in AAA84438 (PubMed:7766993).Curated1
Sequence conflicti558 – 563IRQLNP → NPSAKPS in AAA84438 (PubMed:7766993).Curated6
Sequence conflicti606 – 611VAEVAN → GLKGQ in BAB20265 (Ref. 2) Curated6
Sequence conflicti615 – 622FIRNFPQG → SPEFPPR in AAA84438 (PubMed:7766993).Curated8
Sequence conflicti691R → S in AAA84438 (PubMed:7766993).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_013702150A → S.1 PublicationCorresponds to variant rs4148756dbSNPEnsembl.1
Natural variantiVAR_048133242R → G.Corresponds to variant rs17584642dbSNPEnsembl.1
Natural variantiVAR_035735471R → T in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_031435545D → N.1 PublicationCorresponds to variant rs35698797dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS1580.1.
RefSeqiNP_036221.2. NM_012089.2.
UniGeneiHs.17614.

Genome annotation databases

EnsembliENST00000344517; ENSP00000355637; ENSG00000135776.
GeneIDi23456.
KEGGihsa:23456.
UCSCiuc001htp.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

ABCMdb

Database for mutations in ABC proteins

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS1580.1.
RefSeqiNP_036221.2. NM_012089.2.
UniGeneiHs.17614.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3ZDQX-ray2.85A152-738[»]
4AYTX-ray2.85A152-738[»]
4AYWX-ray3.30A1-738[»]
4AYXX-ray2.90A152-738[»]
ProteinModelPortaliQ9NRK6.
SMRiQ9NRK6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi117020. 25 interactors.
IntActiQ9NRK6. 2 interactors.
STRINGi9606.ENSP00000355637.

Protein family/group databases

TCDBi3.A.1.201.17. the atp-binding cassette (abc) superfamily.

PTM databases

iPTMnetiQ9NRK6.
PhosphoSitePlusiQ9NRK6.
SwissPalmiQ9NRK6.

Polymorphism and mutation databases

BioMutaiABCB10.
DMDMi143811359.

Proteomic databases

EPDiQ9NRK6.
MaxQBiQ9NRK6.
PaxDbiQ9NRK6.
PeptideAtlasiQ9NRK6.
PRIDEiQ9NRK6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000344517; ENSP00000355637; ENSG00000135776.
GeneIDi23456.
KEGGihsa:23456.
UCSCiuc001htp.4. human.

Organism-specific databases

CTDi23456.
DisGeNETi23456.
GeneCardsiABCB10.
H-InvDBHIX0028493.
HGNCiHGNC:41. ABCB10.
HPAiCAB044063.
CAB044065.
HPA055175.
MIMi605454. gene.
neXtProtiNX_Q9NRK6.
OpenTargetsiENSG00000135776.
PharmGKBiPA24385.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0058. Eukaryota.
COG1132. LUCA.
GeneTreeiENSGT00550000074497.
HOVERGENiHBG008358.
InParanoidiQ9NRK6.
KOiK05657.
OMAiWAGDEAW.
OrthoDBiEOG091G0HVA.
PhylomeDBiQ9NRK6.
TreeFamiTF105198.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000135776-MONOMER.
BRENDAi3.6.3.43. 2681.
ReactomeiR-HSA-1369007. Mitochondrial ABC transporters.

Miscellaneous databases

GenomeRNAii23456.
PROiQ9NRK6.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000135776.
CleanExiHS_ABCB10.
GenevisibleiQ9NRK6. HS.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR011527. ABC1_TM_dom.
IPR003439. ABC_transporter-like.
IPR017871. ABC_transporter_CS.
IPR030260. ABCB10.
IPR027417. P-loop_NTPase.
[Graphical view]
PANTHERiPTHR24221:SF121. PTHR24221:SF121. 1 hit.
PfamiPF00664. ABC_membrane. 1 hit.
PF00005. ABC_tran. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
SSF90123. SSF90123. 1 hit.
PROSITEiPS50929. ABC_TM1F. 1 hit.
PS00211. ABC_TRANSPORTER_1. 1 hit.
PS50893. ABC_TRANSPORTER_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiABCBA_HUMAN
AccessioniPrimary (citable) accession number: Q9NRK6
Secondary accession number(s): Q13040, Q6P1Q8, Q9H3V0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: August 2, 2002
Last sequence update: April 3, 2007
Last modified: November 2, 2016
This is version 148 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (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.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.