EMDB/PDB/SASBDBから引用されている文献のデータベース

キーワード
構造解析手法	All X線回折 NMR 電子顕微鏡小角散乱中性子線回折線維回折粉末回折赤外分光 EPR FRET 理論モデルハイブリッド
著者・登録者
ジャーナル
IF	>30 >20 >10 >5 all

タイトル	Structural basis of substrate recognition and translocation by human very long-chain fatty acid transporter ABCD1.
ジャーナル・号・ページ	Nat Commun, Vol. 13, Issue 1, Page 3299, Year 2022
掲載日	2022年6月8日
著者	Zhi-Peng Chen / Da Xu / Liang Wang / Yao-Xu Mao / Yang Li / Meng-Ting Cheng / Cong-Zhao Zhou / Wen-Tao Hou / Yuxing Chen /
PubMed 要旨	Human ABC transporter ABCD1 transports very long-chain fatty acids from cytosol to peroxisome for β-oxidation, dysfunction of which usually causes the X-linked adrenoleukodystrophy (X-ALD). Here, we ...Human ABC transporter ABCD1 transports very long-chain fatty acids from cytosol to peroxisome for β-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.
リンク	Nat Commun / PubMed:35676282 / PubMed Central
手法	EM (単粒子)
解像度	2.8 - 3.59 Å
構造データ	32152 7vwc EMDB-32152, PDB-7vwc: Cryo-EM structure of human very long-chain fatty acid ABC transporter ABCD1 手法: EM (単粒子) / 解像度: 3.53 Å 32171 7vx8 EMDB-32171, PDB-7vx8: Cryo-EM structure of ATP-bound human very long-chain fatty acid ABC transporter ABCD1 手法: EM (単粒子) / 解像度: 2.8 Å 32224 7vzb EMDB-32224, PDB-7vzb: Cryo-EM structure of C22:0-CoA bound human very long-chain fatty acid ABC transporter ABCD1 手法: EM (単粒子) / 解像度: 3.59 Å
化合物	ChemComp-82T: [(2R)-3-[2-azanylethoxy(oxidanyl)phosphoryl]oxy-2-oxidanyl-propyl] octadecanoate / ステアロイルリソホスファチジルエタノ-ルアミン ChemComp-ATP: ADENOSINE-5'-TRIPHOSPHATE / ATP / ATP, エネルギー貯蔵分子YM ChemComp-MG: Unknown entry / マグネシウムジカチオン ChemComp-Y01: CHOLESTEROL HEMISUCCINATE / コレステリルヘミスクシナ-ト ChemComp-FFI*: S-[2-[3-[[(2R)-4-[[[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-oxidanyl-3-phosphonooxy-oxolan-2-yl]methoxy-oxidanyl-phosphoryl]oxy-oxidanyl-phosphoryl]oxy-3,3-dimethyl-2-oxidanyl-butanoyl]amino]propanoylamino]ethyl] docosanethioate
由来	caenorhabditis elegans (センチュウ) homo sapiens (ヒト)
キーワード	TRANSPORT PROTEIN / very long-chain fatty / Peroxisome / ABC transporter