UniProtKB - P48410 (ABCD1_MOUSE)
ATP-binding cassette sub-family D member 1
Abcd1
Functioni
ATP-dependent transporter of the ATP-binding cassette (ABC) family involved in the transport of very long chain fatty acid (VLCFA)-CoA from the cytosol to the peroxisome lumen. Has fatty acyl-CoA thioesterase (ACOT) and ATPase activities. Coupled to the ATP-dependent transporter activity has also a fatty acyl-CoA thioesterase activity (ACOT) and hydrolyzes VLCFA-CoA into VLCFA prior their ATP-dependent transport into peroxisomes, the ACOT activity is essential during this transport process (By similarity).
Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation (PubMed:9126326, PubMed:9418970, PubMed:9256488, PubMed:18854420, PubMed:23123468, PubMed:23604518, PubMed:25255441, PubMed:25583114, PubMed:26108493).
Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance (PubMed:11875044, PubMed:15489218, PubMed:26108493).
Controls also the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization (PubMed:18344354, PubMed:22521832, PubMed:23604518, PubMed:25583114).
And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (PubMed:18723473).
By similarity14 PublicationsCatalytic activityi
- This reaction proceeds in the forwardBy similarity direction.
- a very long-chain fatty acid(in) + ATP + H2O = a very long-chain fatty acid(out) + ADP + H+ + phosphateBy similarityThis reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 507 – 514 | ATPPROSITE-ProRule annotation | 8 |
GO - Molecular functioni
- ABC-type fatty-acyl-CoA transporter activity Source: UniProtKB
- acyl-CoA hydrolase activity Source: UniProtKB
- ADP binding Source: UniProtKB
- ATPase-coupled transmembrane transporter activity Source: UniProtKB
- ATP binding Source: UniProtKB
- ATP hydrolysis activity Source: UniProtKB
- enzyme binding Source: MGI
- identical protein binding Source: MGI
- long-chain fatty acid transporter activity Source: UniProtKB
- protein heterodimerization activity Source: MGI
- protein homodimerization activity Source: MGI
GO - Biological processi
- fatty acid beta-oxidation Source: UniProtKB
- fatty acid elongation Source: UniProtKB
- fatty acid homeostasis Source: UniProtKB
- long-chain fatty acid catabolic process Source: UniProtKB
- long-chain fatty acid import into peroxisome Source: UniProtKB
- myelin maintenance Source: UniProtKB
- negative regulation of cytokine production involved in inflammatory response Source: UniProtKB
- negative regulation of reactive oxygen species biosynthetic process Source: UniProtKB
- neuron projection maintenance Source: UniProtKB
- peroxisome organization Source: MGI
- positive regulation of fatty acid beta-oxidation Source: UniProtKB
- positive regulation of unsaturated fatty acid biosynthetic process Source: UniProtKB
- regulation of cellular response to oxidative stress Source: UniProtKB
- regulation of fatty acid beta-oxidation Source: UniProtKB
- regulation of mitochondrial depolarization Source: UniProtKB
- regulation of oxidative phosphorylation Source: UniProtKB
- sterol homeostasis Source: UniProtKB
- very long-chain fatty acid catabolic process Source: UniProtKB
- very long-chain fatty acid metabolic process Source: UniProtKB
- very long-chain fatty-acyl-CoA catabolic process Source: UniProtKB
Keywordsi
Molecular function | Hydrolase, Translocase |
Biological process | Transport |
Ligand | ATP-binding, Nucleotide-binding |
Enzyme and pathway databases
BRENDAi | 7.6.2.4, 3474 |
Reactomei | R-MMU-1369062, ABC transporters in lipid homeostasis R-MMU-2046105, Linoleic acid (LA) metabolism R-MMU-2046106, alpha-linolenic acid (ALA) metabolism R-MMU-390247, Beta-oxidation of very long chain fatty acids R-MMU-9603798, Class I peroxisomal membrane protein import |
Names & Taxonomyi
Protein namesi | |
Gene namesi | |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:1349215, Abcd1 |
VEuPathDBi | HostDB:ENSMUSG00000031378 |
Subcellular locationi
Endoplasmic reticulum
- Endoplasmic reticulum membrane By similarity; Multi-pass membrane protein By similarity
Mitochondrion
- Mitochondrion membrane By similarity; Multi-pass membrane protein By similarity
Peroxisome
- Peroxisome membrane By similarity; Multi-pass membrane protein Sequence analysis
Lysosome
- Lysosome membrane By similarity; Multi-pass membrane protein By similarity
Endoplasmic reticulum
- endoplasmic reticulum membrane Source: UniProtKB
Lysosome
- lysosomal membrane Source: UniProtKB
Mitochondrion
- mitochondrial membrane Source: UniProtKB
- mitochondrion Source: MGI
Peroxisome
- integral component of peroxisomal membrane Source: MGI
- peroxisomal membrane Source: HGNC-UCL
- peroxisome Source: MGI
Other locations
- cytoplasm Source: MGI
- perinuclear region of cytoplasm Source: MGI
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transmembranei | 92 – 112 | HelicalPROSITE-ProRule annotationAdd BLAST | 21 | |
Transmembranei | 131 – 151 | HelicalPROSITE-ProRule annotationAdd BLAST | 21 | |
Transmembranei | 238 – 258 | HelicalPROSITE-ProRule annotationAdd BLAST | 21 | |
Transmembranei | 338 – 358 | HelicalPROSITE-ProRule annotationAdd BLAST | 21 | |
Transmembranei | 473 – 493 | HelicalPROSITE-ProRule annotationAdd BLAST | 21 |
Keywords - Cellular componenti
Endoplasmic reticulum, Lysosome, Membrane, Mitochondrion, PeroxisomePathology & Biotechi
Disruption phenotypei
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000093305 | 1 – 736 | ATP-binding cassette sub-family D member 1Add BLAST | 736 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 214 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Post-translational modificationi
Keywords - PTMi
GlycoproteinProteomic databases
EPDi | P48410 |
MaxQBi | P48410 |
PaxDbi | P48410 |
PeptideAtlasi | P48410 |
PRIDEi | P48410 |
ProteomicsDBi | 285955 |
PTM databases
GlyGeni | P48410, 1 site |
iPTMneti | P48410 |
PhosphoSitePlusi | P48410 |
Expressioni
Tissue specificityi
Developmental stagei
Inductioni
Gene expression databases
Bgeei | ENSMUSG00000031378, Expressed in bone marrow and 303 other tissues |
ExpressionAtlasi | P48410, baseline and differential |
Genevisiblei | P48410, MM |
Interactioni
Subunit structurei
Can form homodimers and heterodimers with ABCD2 and ABCD3 (PubMed:15276650). Dimerization is necessary to form an active transporter (By similarity). The minimal functional unit is a homodimer but the major oligomeric form in peroxisomal membrane is a homotetramer. Forms heterotramers with ABCD2 (By similarity).
Interacts with PEX19; facilitates ABCD1 insertion into the peroxisome membrane (By similarity).
By similarity1 PublicationBinary interactionsi
P48410
With | #Exp. | IntAct |
---|---|---|
ABCD1 [P33897] from Homo sapiens. | 2 | EBI-81118,EBI-81045 |
GO - Molecular functioni
- enzyme binding Source: MGI
- identical protein binding Source: MGI
- protein heterodimerization activity Source: MGI
- protein homodimerization activity Source: MGI
Protein-protein interaction databases
BioGRIDi | 198062, 2 interactors |
IntActi | P48410, 1 interactor |
STRINGi | 10090.ENSMUSP00000002084 |
Miscellaneous databases
RNActi | P48410, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 94 – 386 | ABC transmembrane type-1PROSITE-ProRule annotationAdd BLAST | 293 | |
Domaini | 474 – 729 | ABC transporterPROSITE-ProRule annotationAdd BLAST | 256 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 67 – 186 | Interaction with PEX19By similarityAdd BLAST | 120 | |
Regioni | 67 – 84 | PEX19 binding site and required for peroxisomal targetingBy similarityAdd BLAST | 18 | |
Regioni | 658 – 736 | Required for homodimerizationBy similarityAdd BLAST | 79 |
Domaini
Sequence similaritiesi
Keywords - Domaini
Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG0064, Eukaryota |
GeneTreei | ENSGT00950000182955 |
HOGENOMi | CLU_007587_1_1_1 |
InParanoidi | P48410 |
OMAi | IPRWNSK |
OrthoDBi | 309052at2759 |
PhylomeDBi | P48410 |
TreeFami | TF105205 |
Family and domain databases
Gene3Di | 3.40.50.300, 1 hit |
InterProi | View protein in InterPro IPR003593, AAA+_ATPase IPR011527, ABC1_TM_dom IPR003439, ABC_transporter-like_ATP-bd IPR017871, ABC_transporter-like_CS IPR031237, ALDP IPR005283, FA_transporter IPR027417, P-loop_NTPase |
PANTHERi | PTHR11384:SF21, PTHR11384:SF21, 1 hit |
Pfami | View protein in Pfam PF06472, ABC_membrane_2, 1 hit PF00005, ABC_tran, 1 hit |
SMARTi | View protein in SMART SM00382, AAA, 1 hit |
SUPFAMi | SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR00954, 3a01203, 1 hit |
PROSITEi | View protein in PROSITE PS50929, ABC_TM1F, 1 hit PS00211, ABC_TRANSPORTER_1, 1 hit PS50893, ABC_TRANSPORTER_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
10 20 30 40 50
MPVLSTPRPS RVTTLKRTAV VLALTAYGVH KIYPLVRQCL TPARGPQVPA
60 70 80 90 100
GEPTQEASGA TATKAGMNRV FLQRLLALLR LLFPRVLCRE TGLLALHSAA
110 120 130 140 150
LVSRTFLSVY VARLDGRLAR CIVRKDPRAF SWQLLQWLLI ALPATFINSA
160 170 180 190 200
IRYLEGQLAL SFRSRLVAHA YGLYFSQQTY YRVSNMDGRL RNPDQSLTED
210 220 230 240 250
VVAFAASVAH LYSNLTKPLL DVAVTSYTLL RAARSRGAGT AWPSAIAGLV
260 270 280 290 300
VFLTANVLRA FSPKFGELVA EEARRKGELR YMHSRVVANS EEIAFYGGHE
310 320 330 340 350
VELALLQHSY QDLASQINLI LLERLWYVML EQFLMKYVWS ASGLLMVAVP
360 370 380 390 400
IITATGYAES DSEAMKKAAL EMKEEELVSE RTEAFTIARN LLTAAADATE
410 420 430 440 450
RIMSSYKEVT ELAGYTARVY EMFQVFEDVK HCRFKRTGDL EEAQAGPGVM
460 470 480 490 500
VQSGVHVEGP LKIQGQVVDV EQGIICENIP IITPTGEVVV ASLNIRVEEG
510 520 530 540 550
MHLLITGPNG CGKSSLFRIL GGLWPTYSGV LYKPPPQRMF YIPQRPYMSV
560 570 580 590 600
GSLRDQVIYP DSAEDMRRKG CSEQQLEAIL GIVHLRHILQ REGGWEAVCD
610 620 630 640 650
WKDVLSGGEK QRIGMARMFY HRPKYALLDE CTSAVSIDVE GKIFQAAKDA
660 670 680 690 700
GIALLSITHR PSLWKYHTHL LQFDGEGGWK FEKLDSAARL SLTEEKQRLE
710 720 730
QQLAGIPKMQ GRLQELRQIL GEAAAPVQPL VPGVPT
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketA2ALN0 | A2ALN0_MOUSE | ATP-binding cassette sub-family D m... | Abcd1 | 504 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | Z33637 mRNA Translation: CAA83917.1 AK088792 mRNA Translation: BAC40574.1 BC011273 mRNA Translation: AAH11273.1 BC079840 mRNA Translation: AAH79840.1 AJ009991 Genomic DNA Translation: CAA08935.1 AF133093 Genomic DNA No translation available. |
CCDSi | CCDS30210.1 |
PIRi | S47044 |
RefSeqi | NP_031461.1, NM_007435.2 |
Genome annotation databases
Ensembli | ENSMUST00000002084; ENSMUSP00000002084; ENSMUSG00000031378 |
GeneIDi | 11666 |
KEGGi | mmu:11666 |
UCSCi | uc009tml.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | Z33637 mRNA Translation: CAA83917.1 AK088792 mRNA Translation: BAC40574.1 BC011273 mRNA Translation: AAH11273.1 BC079840 mRNA Translation: AAH79840.1 AJ009991 Genomic DNA Translation: CAA08935.1 AF133093 Genomic DNA No translation available. |
CCDSi | CCDS30210.1 |
PIRi | S47044 |
RefSeqi | NP_031461.1, NM_007435.2 |
3D structure databases
AlphaFoldDBi | P48410 |
SMRi | P48410 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 198062, 2 interactors |
IntActi | P48410, 1 interactor |
STRINGi | 10090.ENSMUSP00000002084 |
PTM databases
GlyGeni | P48410, 1 site |
iPTMneti | P48410 |
PhosphoSitePlusi | P48410 |
Proteomic databases
EPDi | P48410 |
MaxQBi | P48410 |
PaxDbi | P48410 |
PeptideAtlasi | P48410 |
PRIDEi | P48410 |
ProteomicsDBi | 285955 |
Protocols and materials databases
Antibodypediai | 30940, 422 antibodies from 35 providers |
DNASUi | 11666 |
Genome annotation databases
Ensembli | ENSMUST00000002084; ENSMUSP00000002084; ENSMUSG00000031378 |
GeneIDi | 11666 |
KEGGi | mmu:11666 |
UCSCi | uc009tml.1, mouse |
Organism-specific databases
CTDi | 215 |
MGIi | MGI:1349215, Abcd1 |
VEuPathDBi | HostDB:ENSMUSG00000031378 |
Phylogenomic databases
eggNOGi | KOG0064, Eukaryota |
GeneTreei | ENSGT00950000182955 |
HOGENOMi | CLU_007587_1_1_1 |
InParanoidi | P48410 |
OMAi | IPRWNSK |
OrthoDBi | 309052at2759 |
PhylomeDBi | P48410 |
TreeFami | TF105205 |
Enzyme and pathway databases
BRENDAi | 7.6.2.4, 3474 |
Reactomei | R-MMU-1369062, ABC transporters in lipid homeostasis R-MMU-2046105, Linoleic acid (LA) metabolism R-MMU-2046106, alpha-linolenic acid (ALA) metabolism R-MMU-390247, Beta-oxidation of very long chain fatty acids R-MMU-9603798, Class I peroxisomal membrane protein import |
Miscellaneous databases
BioGRID-ORCSi | 11666, 1 hit in 61 CRISPR screens |
ChiTaRSi | Abcd1, mouse |
PROi | PR:P48410 |
RNActi | P48410, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000031378, Expressed in bone marrow and 303 other tissues |
ExpressionAtlasi | P48410, baseline and differential |
Genevisiblei | P48410, MM |
Family and domain databases
Gene3Di | 3.40.50.300, 1 hit |
InterProi | View protein in InterPro IPR003593, AAA+_ATPase IPR011527, ABC1_TM_dom IPR003439, ABC_transporter-like_ATP-bd IPR017871, ABC_transporter-like_CS IPR031237, ALDP IPR005283, FA_transporter IPR027417, P-loop_NTPase |
PANTHERi | PTHR11384:SF21, PTHR11384:SF21, 1 hit |
Pfami | View protein in Pfam PF06472, ABC_membrane_2, 1 hit PF00005, ABC_tran, 1 hit |
SMARTi | View protein in SMART SM00382, AAA, 1 hit |
SUPFAMi | SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR00954, 3a01203, 1 hit |
PROSITEi | View protein in PROSITE PS50929, ABC_TM1F, 1 hit PS00211, ABC_TRANSPORTER_1, 1 hit PS50893, ABC_TRANSPORTER_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | ABCD1_MOUSE | |
Accessioni | P48410Primary (citable) accession number: P48410 Secondary accession number(s): Q9QY41, Q9QZ32 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 1, 1996 |
Last sequence update: | February 1, 1996 | |
Last modified: | May 25, 2022 | |
This is version 182 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families