登録情報 データベース : EMDB / ID : EMD-6940 構造の表示 ダウンロードとリンクタイトル Structure of human mitochondrial trifunctional protein, tetramer マップデータ 詳細 試料複合体 : human mitochondrial trifunctional proteinタンパク質・ペプチド : Trifunctional enzyme subunit alpha, mitochondrialタンパク質・ペプチド : Trifunctional enzyme subunit beta, mitochondrial 詳細 キーワード fatty acid beta-oxidation / cryo-EM single-particle reconstruction / mitochondrial trifunctional protein / LYASE / HYDROLASE-TRANSFERASE complex機能・相同性 機能・相同性情報分子機能 ドメイン・相同性 構成要素
long-chain-3-hydroxyacyl-CoA dehydrogenase / cardiolipin acyl-chain remodeling / Acyl chain remodeling of CL / Beta oxidation of myristoyl-CoA to lauroyl-CoA / Beta oxidation of palmitoyl-CoA to myristoyl-CoA / acetyl-CoA C-myristoyltransferase / acetyl-CoA C-myristoyltransferase activity / mitochondrial fatty acid beta-oxidation multienzyme complex / 3-hydroxyacyl-CoA dehydratase activity / mitochondrial fatty acid beta-oxidation of unsaturated fatty acids ... long-chain-3-hydroxyacyl-CoA dehydrogenase / cardiolipin acyl-chain remodeling / Acyl chain remodeling of CL / Beta oxidation of myristoyl-CoA to lauroyl-CoA / Beta oxidation of palmitoyl-CoA to myristoyl-CoA / acetyl-CoA C-myristoyltransferase / acetyl-CoA C-myristoyltransferase activity / mitochondrial fatty acid beta-oxidation multienzyme complex / 3-hydroxyacyl-CoA dehydratase activity / mitochondrial fatty acid beta-oxidation of unsaturated fatty acids / Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA / Beta oxidation of hexanoyl-CoA to butanoyl-CoA / Beta oxidation of octanoyl-CoA to hexanoyl-CoA / Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA / acetyl-CoA C-acyltransferase / acetyl-CoA C-acyltransferase activity / long-chain-3-hydroxyacyl-CoA dehydrogenase activity / enoyl-CoA hydratase / acetyl-CoA C-acetyltransferase activity / 3-hydroxyacyl-CoA dehydrogenase activity / fatty-acyl-CoA binding / enoyl-CoA hydratase activity / mitochondrial envelope / lncRNA binding / fatty acid beta-oxidation / mitochondrial nucleoid / NAD+ binding / 転移酵素; アシル基を移すもの; アミノアシル基以外のアシル基を移すもの / response to insulin / gene expression / cellular response to lipopolysaccharide / mitochondrial outer membrane / mitochondrial inner membrane / response to xenobiotic stimulus / protein-containing complex binding / endoplasmic reticulum / mitochondrion / RNA binding / cytosol 類似検索 - 分子機能 Fatty acid oxidation complex, alpha subunit, mitochondrial / 3-hydroxyacyl-CoA dehydrogenase, conserved site / 3-hydroxyacyl-CoA dehydrogenase signature. / 3-hydroxyacyl-CoA dehydrogenase, C-terminal / 3-hydroxyacyl-CoA dehydrogenase, NAD binding / 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain / 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain / Thiolase, active site / Thiolases active site. / Thiolase, acyl-enzyme intermediate active site ... Fatty acid oxidation complex, alpha subunit, mitochondrial / 3-hydroxyacyl-CoA dehydrogenase, conserved site / 3-hydroxyacyl-CoA dehydrogenase signature. / 3-hydroxyacyl-CoA dehydrogenase, C-terminal / 3-hydroxyacyl-CoA dehydrogenase, NAD binding / 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain / 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain / Thiolase, active site / Thiolases active site. / Thiolase, acyl-enzyme intermediate active site / Thiolases acyl-enzyme intermediate signature. / Thiolase, conserved site / Thiolases signature 2. / Enoyl-CoA hydratase/isomerase, conserved site / Enoyl-CoA hydratase/isomerase signature. / Thiolase / Thiolase, C-terminal / Thiolase, C-terminal domain / Thiolase, N-terminal / Thiolase, N-terminal domain / Enoyl-CoA hydratase/isomerase / Enoyl-CoA hydratase/isomerase / 6-phosphogluconate dehydrogenase-like, C-terminal domain superfamily / ClpP/crotonase-like domain superfamily / Thiolase-like / NAD(P)-binding domain superfamily 類似検索 - ドメイン・相同性 Trifunctional enzyme subunit alpha, mitochondrial / Trifunctional enzyme subunit beta, mitochondrial 類似検索 - 構成要素生物種 Homo sapiens (ヒト)手法 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度 : 4.2 Å 詳細 データ登録者Liang K / Li N / Dai J / Wang X / Liu P / Chen X / Wang C / Gao N / Xiao J 引用ジャーナル : Proc Natl Acad Sci U S A / 年 : 2018タイトル : Cryo-EM structure of human mitochondrial trifunctional protein.著者 : Kai Liang / Ningning Li / Xiao Wang / Jianye Dai / Pulan Liu / Chu Wang / Xiao-Wei Chen / Ning Gao / Junyu Xiao / 要旨 : The mitochondrial trifunctional protein (TFP) catalyzes three reactions in the fatty acid β-oxidation process. Mutations in the two TFP subunits cause mitochondrial trifunctional protein deficiency ... The mitochondrial trifunctional protein (TFP) catalyzes three reactions in the fatty acid β-oxidation process. Mutations in the two TFP subunits cause mitochondrial trifunctional protein deficiency and acute fatty liver of pregnancy that can lead to death. Here we report a 4.2-Å cryo-electron microscopy α2β2 tetrameric structure of the human TFP. The tetramer has a V-shaped architecture that displays a distinct assembly compared with the bacterial TFPs. A concave surface of the TFP tetramer interacts with the detergent molecules in the structure, suggesting that this region is involved in associating with the membrane. Deletion of a helical hairpin in TFPβ decreases its binding to the liposomes in vitro and reduces its membrane targeting in cells. Our results provide the structural basis for TFP function and have important implications for fatty acid oxidation related diseases. 履歴 登録 2018年4月20日 - ヘッダ(付随情報) 公開 2018年6月20日 - マップ公開 2018年6月20日 - 更新 2024年3月27日 - 現状 2024年3月27日 処理サイト : PDBj / 状態 : 公開
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