7vwc

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Cryo-EM structure of human very long-chain fatty acid ABC transporter ABCD1

Structural highlights

7vwc is a 2 chain structure. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Ligands:82T
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Publication Abstract from PubMed

Human ABC transporter ABCD1 transports very long-chain fatty acids from cytosol to peroxisome for beta-oxidation, dysfunction of which usually causes the X-linked adrenoleukodystrophy (X-ALD). Here, we report three cryogenic electron microscopy structures of ABCD1: the apo-form, substrate- and ATP-bound forms. Distinct from what was seen in the previously reported ABC transporters, the two symmetric molecules of behenoyl coenzyme A (C22:0-CoA) cooperatively bind to the transmembrane domains (TMDs). For each C22:0-CoA, the hydrophilic 3'-phospho-ADP moiety of CoA portion inserts into one TMD, with the succeeding pantothenate and cysteamine moiety crossing the inter-domain cavity, whereas the hydrophobic fatty acyl chain extends to the opposite TMD. Structural analysis combined with biochemical assays illustrates snapshots of ABCD1-mediated substrate transport cycle. It advances our understanding on the selective oxidation of fatty acids and molecular pathology of X-ALD.

Structural basis of substrate recognition and translocation by human very long-chain fatty acid transporter ABCD1.,Chen ZP, Xu D, Wang L, Mao YX, Li Y, Cheng MT, Zhou CZ, Hou WT, Chen Y Nat Commun. 2022 Jun 8;13(1):3299. doi: 10.1038/s41467-022-30974-5. PMID:35676282[1]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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Citations
2 reviews cite this structure
Kumar et al. (2024)
No citations found

References

  1. Chen ZP, Xu D, Wang L, Mao YX, Li Y, Cheng MT, Zhou CZ, Hou WT, Chen Y. Structural basis of substrate recognition and translocation by human very long-chain fatty acid transporter ABCD1. Nat Commun. 2022 Jun 8;13(1):3299. doi: 10.1038/s41467-022-30974-5. PMID:35676282 doi:http://dx.doi.org/10.1038/s41467-022-30974-5

Contents


PDB ID 7vwc

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