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7shm

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(New page: '''Unreleased structure''' The entry 7shm is ON HOLD until Paper Publication Authors: Description: Category: Unreleased Structures)
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'''Unreleased structure'''
 
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The entry 7shm is ON HOLD until Paper Publication
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==Cryo-EM structure of ATP-bound human peroxisomal fatty acid transporter ABCD1==
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<StructureSection load='7shm' size='340' side='right'caption='[[7shm]], [[Resolution|resolution]] 3.14&Aring;' scene=''>
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Authors:
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== Structural highlights ==
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<table><tr><td colspan='2'>[[7shm]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7SHM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7SHM FirstGlance]. <br>
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Description:
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ATP:ADENOSINE-5-TRIPHOSPHATE'>ATP</scene></td></tr>
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[[Category: Unreleased Structures]]
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/ABC-type_fatty-acyl-CoA_transporter ABC-type fatty-acyl-CoA transporter], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=7.6.2.4 7.6.2.4] </span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=7shm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7shm OCA], [https://pdbe.org/7shm PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7shm RCSB], [https://www.ebi.ac.uk/pdbsum/7shm PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7shm ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[[https://www.uniprot.org/uniprot/ABCD1_HUMAN ABCD1_HUMAN]] X-linked cerebral adrenoleukodystrophy;Adrenomyeloneuropathy;CADDS. The disease is caused by variants affecting the gene represented in this entry. The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31.<ref>PMID:11992258</ref>
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== Function ==
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[[https://www.uniprot.org/uniprot/ABCD1_HUMAN ABCD1_HUMAN]] 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 (PubMed:11248239, PubMed:15682271, PubMed:16946495, PubMed:18757502, PubMed:21145416, PubMed:23671276, PubMed:29397936, PubMed:33500543). 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 (PubMed:33500543, PubMed:29397936). 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:23671276, PubMed:21145416). 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. Controls also the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (By similarity).[UniProtKB:P48410]<ref>PMID:11248239</ref> <ref>PMID:15682271</ref> <ref>PMID:16946495</ref> <ref>PMID:18757502</ref> <ref>PMID:21145416</ref> <ref>PMID:23671276</ref> <ref>PMID:29397936</ref> <ref>PMID:33500543</ref>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: ABC-type fatty-acyl-CoA transporter]]
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[[Category: Large Structures]]
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[[Category: Li, X]]
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[[Category: Wang, R]]
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[[Category: Abc transporter]]
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[[Category: Abcd]]
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[[Category: Acyl-coa transport]]
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[[Category: Lipid transport]]

Current revision

Cryo-EM structure of ATP-bound human peroxisomal fatty acid transporter ABCD1

PDB ID 7shm

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