+データを開く
-基本情報
登録情報 | データベース: EMDB / ID: EMD-0901 | |||||||||
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タイトル | Structure of N-terminal and C-terminal domains of FANCA | |||||||||
マップデータ | ||||||||||
試料 |
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キーワード | nuclear localization / fanconi anemia core protein / fanconi anemia complementation group a / interstrand crosslink repair / DNA REPAIR | |||||||||
生物種 | Xenopus laevis (アフリカツメガエル) | |||||||||
手法 | 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度: 4.84 Å | |||||||||
データ登録者 | Jeong E / Lee S | |||||||||
資金援助 | 韓国, 2件
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引用 | ジャーナル: Nucleic Acids Res / 年: 2020 タイトル: Structural basis of the fanconi anemia-associated mutations within the FANCA and FANCG complex. 著者: Eunyoung Jeong / Seong-Gyu Lee / Hyun-Suk Kim / Jihyeon Yang / Jinwoo Shin / Youngran Kim / Jihan Kim / Orlando D Schärer / Youngjin Kim / Jung-Eun Yeo / Ho Min Kim / Yunje Cho / 要旨: Monoubiquitination of the Fanconi anemia complementation group D2 (FANCD2) protein by the FA core ubiquitin ligase complex is the central event in the FA pathway. FANCA and FANCG play major roles in ...Monoubiquitination of the Fanconi anemia complementation group D2 (FANCD2) protein by the FA core ubiquitin ligase complex is the central event in the FA pathway. FANCA and FANCG play major roles in the nuclear localization of the FA core complex. Mutations of these two genes are the most frequently observed genetic alterations in FA patients, and most point mutations in FANCA are clustered in the C-terminal domain (CTD). To understand the basis of the FA-associated FANCA mutations, we determined the cryo-electron microscopy (EM) structures of Xenopus laevis FANCA alone at 3.35 Å and 3.46 Å resolution and two distinct FANCA-FANCG complexes at 4.59 and 4.84 Å resolution, respectively. The FANCA CTD adopts an arc-shaped solenoid structure that forms a pseudo-symmetric dimer through its outer surface. FA- and cancer-associated point mutations are widely distributed over the CTD. The two different complex structures capture independent interactions of FANCG with either FANCA C-terminal HEAT repeats, or the N-terminal region. We show that mutations that disturb either of these two interactions prevent the nuclear localization of FANCA, thereby leading to an FA pathway defect. The structure provides insights into the function of FANCA CTD, and provides a framework for understanding FA- and cancer-associated mutations. | |||||||||
履歴 |
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-構造の表示
ムービー |
ムービービューア |
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構造ビューア | EMマップ: SurfViewMolmilJmol/JSmol |
添付画像 |
-ダウンロードとリンク
-EMDBアーカイブ
マップデータ | emd_0901.map.gz | 5.3 MB | EMDBマップデータ形式 | |
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ヘッダ (付随情報) | emd-0901-v30.xml emd-0901.xml | 17.9 KB 17.9 KB | 表示 表示 | EMDBヘッダ |
画像 | emd_0901.png | 13 KB | ||
Filedesc metadata | emd-0901.cif.gz | 4.4 KB | ||
アーカイブディレクトリ | http://ftp.pdbj.org/pub/emdb/structures/EMD-0901 ftp://ftp.pdbj.org/pub/emdb/structures/EMD-0901 | HTTPS FTP |
-検証レポート
文書・要旨 | emd_0901_validation.pdf.gz | 352.9 KB | 表示 | EMDB検証レポート |
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文書・詳細版 | emd_0901_full_validation.pdf.gz | 352.4 KB | 表示 | |
XML形式データ | emd_0901_validation.xml.gz | 6.2 KB | 表示 | |
CIF形式データ | emd_0901_validation.cif.gz | 7 KB | 表示 | |
アーカイブディレクトリ | https://ftp.pdbj.org/pub/emdb/validation_reports/EMD-0901 ftp://ftp.pdbj.org/pub/emdb/validation_reports/EMD-0901 | HTTPS FTP |
-関連構造データ
-リンク
EMDBのページ | EMDB (EBI/PDBe) / EMDataResource |
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-マップ
ファイル | ダウンロード / ファイル: emd_0901.map.gz / 形式: CCP4 / 大きさ: 64 MB / タイプ: IMAGE STORED AS FLOATING POINT NUMBER (4 BYTES) | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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投影像・断面図 | 画像のコントロール
画像は Spider により作成 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
ボクセルのサイズ | X=Y=Z: 1.4 Å | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
密度 |
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対称性 | 空間群: 1 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
詳細 | EMDB XML:
CCP4マップ ヘッダ情報:
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-添付データ
-試料の構成要素
-全体 : FANCA
全体 | 名称: FANCA |
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要素 |
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-超分子 #1: FANCA
超分子 | 名称: FANCA / タイプ: complex / ID: 1 / 親要素: 0 / 含まれる分子: all / 詳細: homo dimer |
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由来(天然) | 生物種: Xenopus laevis (アフリカツメガエル) |
分子量 | 理論値: 500 kDa/nm |
-分子 #1: Fanconi anemia complementation group A
分子 | 名称: Fanconi anemia complementation group A / タイプ: protein_or_peptide / ID: 1 / コピー数: 3 / 光学異性体: LEVO |
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由来(天然) | 生物種: Xenopus laevis (アフリカツメガエル) |
分子量 | 理論値: 119.163812 KDa |
組換発現 | 生物種: Trichoplusia ni (イラクサキンウワバ) |
配列 | 文字列: (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK) ...文字列: (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) (UNK)(UNK) (UNK)(UNK)(UNK)(UNK)(UNK)(UNK)(UNK) 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-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 |
-試料調製
濃度 | 0.8 mg/mL |
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緩衝液 | pH: 8 |
凍結 | 凍結剤: ETHANE |
-電子顕微鏡法
顕微鏡 | FEI TITAN KRIOS |
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撮影 | #0 - Image recording ID: 1 #0 - フィルム・検出器のモデル: FEI FALCON III (4k x 4k) #0 - 平均電子線量: 30.0 e/Å2 / #1 - Image recording ID: 2 #1 - フィルム・検出器のモデル: FEI FALCON III (4k x 4k) #1 - 平均電子線量: 30.0 e/Å2 / #2 - Image recording ID: 3 #2 - フィルム・検出器のモデル: FEI FALCON III (4k x 4k) #2 - 平均電子線量: 30.0 e/Å2 / #3 - Image recording ID: 4 #3 - フィルム・検出器のモデル: FEI FALCON III (4k x 4k) #3 - 平均電子線量: 30.0 e/Å2 |
電子線 | 加速電圧: 300 kV / 電子線源: FIELD EMISSION GUN |
電子光学系 | 照射モード: FLOOD BEAM / 撮影モード: DIFFRACTION |
実験機器 | モデル: Titan Krios / 画像提供: FEI Company |