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 insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4 mouse.
ジャーナル・号・ページ	EMBO J, Vol. 43, Issue 2, Page 225-249, Year 2024
掲載日	2024年1月2日
著者	Zhan Yin / Ahmed-Noor A Agip / Hannah R Bridges / Judy Hirst /
PubMed 要旨	Respiratory complex I (NADH:ubiquinone oxidoreductase) is essential for cellular energy production and NAD homeostasis. Complex I mutations cause neuromuscular, mitochondrial diseases, such as Leigh ...Respiratory complex I (NADH:ubiquinone oxidoreductase) is essential for cellular energy production and NAD homeostasis. Complex I mutations cause neuromuscular, mitochondrial diseases, such as Leigh Syndrome, but their molecular-level consequences remain poorly understood. Here, we use a popular complex I-linked mitochondrial disease model, the ndufs4 mouse, to define the structural, biochemical, and functional consequences of the absence of subunit NDUFS4. Cryo-EM analyses of the complex I from ndufs4 mouse hearts revealed a loose association of the NADH-dehydrogenase module, and discrete classes containing either assembly factor NDUFAF2 or subunit NDUFS6. Subunit NDUFA12, which replaces its paralogue NDUFAF2 in mature complex I, is absent from all classes, compounding the deletion of NDUFS4 and preventing maturation of an NDUFS4-free enzyme. We propose that NDUFAF2 recruits the NADH-dehydrogenase module during assembly of the complex. Taken together, the findings provide new molecular-level understanding of the ndufs4 mouse model and complex I-linked mitochondrial disease.
リンク	EMBO J / PubMed:38177503 / PubMed Central
手法	EM (単粒子)
解像度	3.2 - 6.2 Å
構造データ	16398 8c2s EMDB-16398, PDB-8c2s: Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart (Class 1). 手法: EM (単粒子) / 解像度: 3.9 Å EMDB-16514: Cryo-EM structure of NDUFS4 knockout of respiratory complex I from Mus musculus kidney. 手法: EM (単粒子) / 解像度: 6.2 Å 16515 8ca1 EMDB-16515, PDB-8ca1: Cryo-EM structure of the ACADVL dimer from Mus musculus. 手法: EM (単粒子) / 解像度: 4.3 Å 16516 8ca3 EMDB-16516, PDB-8ca3: Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart (Class 2). 手法: EM (単粒子) / 解像度: 3.2 Å 16517 8ca4 EMDB-16517, PDB-8ca4: Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart (Class 2 N-domain). 手法: EM (単粒子) / 解像度: 3.25 Å 16518 8ca5 EMDB-16518, PDB-8ca5: Cryo-EM structure NDUFS4 knockout complex I from Mus musculus heart (Class 3). 手法: EM (単粒子) / 解像度: 3.9 Å
化合物	ChemComp-SF4: IRON/SULFUR CLUSTER ChemComp-PC1: 1,2-DIACYL-SN-GLYCERO-3-PHOSPHOCHOLINE / リン脂質YM ChemComp-FES: FE2/S2 (INORGANIC) CLUSTER ChemComp-FMN: FLAVIN MONONUCLEOTIDE / FMN ChemComp-3PE: 1,2-Distearoyl-sn-glycerophosphoethanolamine / DSPE / リン脂質YM ChemComp-CDL: CARDIOLIPIN / リン脂質YM ChemComp-LMT: DODECYL-BETA-D-MALTOSIDE / ドデシルβ-D-マルトシド / 可溶化剤YM ChemComp-GTP: GUANOSINE-5'-TRIPHOSPHATE / GTP / GTP, エネルギー貯蔵分子YM ChemComp-NAP: NADP NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATE ChemComp-ZN: Unknown entry ChemComp-EHZ: ~{S}-[2-[3-[[(2~{R})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butanoyl]amino]propanoylamino]ethyl] (3~{S})-3-oxidanyltetradecanethioate ChemComp-FAD: FLAVIN-ADENINE DINUCLEOTIDE / FAD / FADYM ChemComp-UQ9: Ubiquinone-9 ChemComp-NDP: NADPH DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATE / NADPH
由来	mus musculus (ハツカネズミ)
キーワード	OXIDOREDUCTASE / NADH ubiquinone oxidoreductase / Complex I