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データを開く
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基本情報
| 登録情報 | ![]() | |||||||||
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| タイトル | Formation of I2+III2 supercomplex rescues respiratory chain defects | |||||||||
マップデータ | Composite map | |||||||||
試料 |
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キーワード | Super complex XL (CI2+CIII2) / ELECTRON TRANSPORT / Translocase | |||||||||
| 機能・相同性 | 機能・相同性情報response to D-galactosamine / Complex III assembly / response to cobalamin / Mitochondrial protein import / response to injury involved in regulation of muscle adaptation / Protein lipoylation / Mitochondrial Fatty Acid Beta-Oxidation / Complex I biogenesis / RHOG GTPase cycle / Mitochondrial ribosome-associated quality control ...response to D-galactosamine / Complex III assembly / response to cobalamin / Mitochondrial protein import / response to injury involved in regulation of muscle adaptation / Protein lipoylation / Mitochondrial Fatty Acid Beta-Oxidation / Complex I biogenesis / RHOG GTPase cycle / Mitochondrial ribosome-associated quality control / response to mercury ion / Mitochondrial translation termination / Respiratory electron transport / subthalamus development / pons development / protein insertion into mitochondrial inner membrane / cerebellar Purkinje cell layer development / mitochondrial respiratory chain complex III assembly / blastocyst hatching / respiratory system process / psychomotor behavior / pyramidal neuron development / Mitochondrial protein degradation / thalamus development / response to light intensity / protein lipoylation / response to alkaloid / mesenchymal stem cell proliferation / cellular response to oxygen levels / iron-sulfur cluster assembly complex / reproductive system development / ubiquinone biosynthetic process / mitochondrial large ribosomal subunit binding / mitochondrial [2Fe-2S] assembly complex / respiratory chain complex / gliogenesis / mesenchymal stem cell differentiation / circulatory system development / negative regulation of non-canonical NF-kappaB signal transduction / respiratory chain complex III / cellular respiration / response to glucagon / quinol-cytochrome-c reductase / response to hydroperoxide / adult walking behavior / cardiac muscle tissue development / neural precursor cell proliferation / oxygen sensor activity / [2Fe-2S] cluster assembly / quinol-cytochrome-c reductase activity / positive regulation of mitochondrial membrane potential / cellular response to glucocorticoid stimulus / stem cell division / iron-sulfur cluster assembly / mitochondrial electron transport, ubiquinol to cytochrome c / response to copper ion / hypothalamus development / midbrain development / NADH:ubiquinone reductase (H+-translocating) / dopamine metabolic process / ubiquinone binding / adult behavior / mitochondrial ATP synthesis coupled electron transport / positive regulation of ATP biosynthetic process / proton motive force-driven mitochondrial ATP synthesis / electron transport coupled proton transport / mitochondrial electron transport, NADH to ubiquinone / acyl binding / mitochondrial respiratory chain complex I assembly / oxidoreductase activity, acting on NAD(P)H / animal organ regeneration / NADH dehydrogenase activity / respiratory chain complex I / response to hyperoxia / positive regulation of execution phase of apoptosis / NADH dehydrogenase (ubiquinone) activity / response to cadmium ion / acyl carrier activity / response to cAMP / neuron development / quinone binding / ATP synthesis coupled electron transport / cellular response to interferon-beta / tricarboxylic acid cycle / negative regulation of reactive oxygen species biosynthetic process / extrinsic apoptotic signaling pathway / cellular response to retinoic acid / Neutrophil degranulation / response to hormone / visual perception / reactive oxygen species metabolic process / neurogenesis / ionotropic glutamate receptor binding / muscle contraction / cerebellum development / aerobic respiration / response to activity / regulation of mitochondrial membrane potential / respiratory electron transport chain / response to nicotine 類似検索 - 分子機能 | |||||||||
| 生物種 | ![]() | |||||||||
| 手法 | 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度: 3.7 Å | |||||||||
データ登録者 | Letts JA / Padavannil A | |||||||||
| 資金援助 | 米国, 1件
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引用 | ジャーナル: Cell Metab / 年: 2025タイトル: Formation of I+III supercomplex rescues respiratory chain defects. 著者: Chao Liang / Abhilash Padavannil / Shan Zhang / Sheryl Beh / David R L Robinson / Jana Meisterknecht / Alfredo Cabrera-Orefice / Timothy R Koves / Chika Watanabe / Miyuki Watanabe / María ...著者: Chao Liang / Abhilash Padavannil / Shan Zhang / Sheryl Beh / David R L Robinson / Jana Meisterknecht / Alfredo Cabrera-Orefice / Timothy R Koves / Chika Watanabe / Miyuki Watanabe / María Illescas / Radiance Lim / Jordan M Johnson / Shuxun Ren / Ya-Jun Wu / Dennis Kappei / Anna Maria Ghelli / Katsuhiko Funai / Hitoshi Osaka / Deborah Muoio / Cristina Ugalde / Ilka Wittig / David A Stroud / James A Letts / Lena Ho / ![]() 要旨: Mitochondrial electron transport chain (ETC) complexes partition between free complexes and quaternary assemblies known as supercomplexes (SCs). However, the physiological requirement for SCs and the ...Mitochondrial electron transport chain (ETC) complexes partition between free complexes and quaternary assemblies known as supercomplexes (SCs). However, the physiological requirement for SCs and the mechanisms regulating their formation remain controversial. Here, we show that genetic perturbations in mammalian ETC complex III (CIII) biogenesis stimulate the formation of a specialized extra-large SC (SC-XL) with a structure of I+III, resolved at 3.7 Å by cryoelectron microscopy (cryo-EM). SC-XL formation increases mitochondrial cristae density, reduces CIII reactive oxygen species (ROS), and sustains normal respiration despite a 70% reduction in CIII activity, effectively rescuing CIII deficiency. Consequently, inhibiting SC-XL formation in CIII mutants using the Uqcrc1 contact site mutation leads to respiratory decompensation. Lastly, SC-XL formation promotes fatty acid oxidation (FAO) and protects against ischemic heart failure in mice. Our study uncovers an unexpected plasticity in the mammalian ETC, where structural adaptations mitigate intrinsic perturbations, and suggests that manipulating SC-XL formation is a potential therapeutic strategy for mitochondrial dysfunction. | |||||||||
| 履歴 |
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構造の表示
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ダウンロードとリンク
-EMDBアーカイブ
| マップデータ | emd_42122.map.gz | 103.8 MB | EMDBマップデータ形式 | |
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| ヘッダ (付随情報) | emd-42122-v30.xml emd-42122.xml | 82.1 KB 82.1 KB | 表示 表示 | EMDBヘッダ |
| FSC (解像度算出) | emd_42122_fsc.xml | 22.4 KB | 表示 | FSCデータファイル |
| 画像 | emd_42122.png | 151.7 KB | ||
| Filedesc metadata | emd-42122.cif.gz | 16.4 KB | ||
| アーカイブディレクトリ | http://ftp.pdbj.org/pub/emdb/structures/EMD-42122 ftp://ftp.pdbj.org/pub/emdb/structures/EMD-42122 | HTTPS FTP |
-関連構造データ
| 関連構造データ | ![]() 8ucaMC C: 同じ文献を引用 ( M: このマップから作成された原子モデル |
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| 類似構造データ | 類似検索 - 機能・相同性 F&H 検索 |
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リンク
| EMDBのページ | EMDB (EBI/PDBe) / EMDataResource |
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| 「今月の分子」の関連する項目 |
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マップ
| ファイル | ダウンロード / ファイル: emd_42122.map.gz / 形式: CCP4 / 大きさ: 113 MB / タイプ: IMAGE STORED AS FLOATING POINT NUMBER (4 BYTES) | ||||||||||||||||||||||||||||||||||||
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| 注釈 | Composite map | ||||||||||||||||||||||||||||||||||||
| 投影像・断面図 | 画像のコントロール
画像は Spider により作成 これらの図は立方格子座標系で作成されたものです | ||||||||||||||||||||||||||||||||||||
| ボクセルのサイズ | X=Y=Z: 0.88 Å | ||||||||||||||||||||||||||||||||||||
| 密度 |
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| 対称性 | 空間群: 1 | ||||||||||||||||||||||||||||||||||||
| 詳細 | EMDB XML:
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-添付データ
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試料の構成要素
+全体 : Mitochondrial Super-complex with CI dimer and CIII dimer (Super-c...
+超分子 #1: Mitochondrial Super-complex with CI dimer and CIII dimer (Super-c...
+分子 #1: NADH-ubiquinone oxidoreductase chain 3
+分子 #2: NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
+分子 #3: NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
+分子 #4: NADH-ubiquinone oxidoreductase chain 1
+分子 #5: NADH-ubiquinone oxidoreductase chain 6
+分子 #6: NADH-ubiquinone oxidoreductase chain 4L
+分子 #7: NADH-ubiquinone oxidoreductase chain 5
+分子 #8: NADH-ubiquinone oxidoreductase chain 4
+分子 #9: NADH-ubiquinone oxidoreductase chain 2
+分子 #10: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mi...
+分子 #11: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mit...
+分子 #12: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
+分子 #13: NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
+分子 #14: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2
+分子 #15: Acyl carrier protein, mitochondrial
+分子 #16: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5
+分子 #17: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
+分子 #18: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8
+分子 #19: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11
+分子 #20: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13
+分子 #21: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
+分子 #22: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3
+分子 #23: NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial
+分子 #24: NADH dehydrogenase [ubiquinone] 1 subunit C2
+分子 #25: NADH dehydrogenase [ubiquinone] iron-sulfur protein 5
+分子 #26: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1
+分子 #27: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mit...
+分子 #28: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mito...
+分子 #29: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6
+分子 #30: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mito...
+分子 #31: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
+分子 #32: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mito...
+分子 #33: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4
+分子 #34: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9
+分子 #35: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7
+分子 #36: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10
+分子 #37: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
+分子 #38: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7
+分子 #39: NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial
+分子 #40: Cytochrome b-c1 complex subunit 1, mitochondrial
+分子 #41: Cytochrome b-c1 complex subunit 2, mitochondrial
+分子 #42: Cytochrome b
+分子 #43: Cytochrome c1, heme protein, mitochondrial
+分子 #44: Cytochrome b-c1 complex subunit 9
+分子 #45: Cytochrome b-c1 complex subunit 7
+分子 #46: Cytochrome b-c1 complex subunit 8
+分子 #47: Cytochrome b-c1 complex subunit 6, mitochondrial
+分子 #48: Cytochrome b-c1 complex subunit 9
+分子 #49: Cytochrome b-c1 complex subunit 10
+分子 #50: Cytochrome b-c1 complex subunit Rieske, mitochondrial
+分子 #51: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
+分子 #52: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial
+分子 #53: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
+分子 #54: NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
+分子 #55: NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial
+分子 #56: MAGNESIUM ION
+分子 #57: 2'-DEOXYGUANOSINE-5'-TRIPHOSPHATE
+分子 #58: NADPH DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATE
+分子 #59: ZINC ION
+分子 #60: ~{S}-[2-[3-[[(2~{R})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butan...
+分子 #61: PROTOPORPHYRIN IX CONTAINING FE
+分子 #62: HEME C
+分子 #63: FLAVIN MONONUCLEOTIDE
+分子 #64: IRON/SULFUR CLUSTER
+分子 #65: FE2/S2 (INORGANIC) CLUSTER
-実験情報
-構造解析
| 手法 | クライオ電子顕微鏡法 |
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解析 | 単粒子再構成法 |
| 試料の集合状態 | particle |
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試料調製
| 緩衝液 | pH: 7.7 |
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| 凍結 | 凍結剤: ETHANE |
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電子顕微鏡法
| 顕微鏡 | TFS GLACIOS |
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| 撮影 | フィルム・検出器のモデル: GATAN K3 (6k x 4k) / 平均電子線量: 60.0 e/Å2 |
| 電子線 | 加速電圧: 200 kV / 電子線源: FIELD EMISSION GUN |
| 電子光学系 | 照射モード: OTHER / 撮影モード: BRIGHT FIELD / 最大 デフォーカス(公称値): 3.0 µm / 最小 デフォーカス(公称値): 0.5 µm |
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コントローラー
万見について




キーワード
データ登録者
米国, 1件
引用
































X (Sec.)
Y (Row.)
Z (Col.)




























解析
FIELD EMISSION GUN

