+データを開く
-基本情報
登録情報 | データベース: PDB / ID: 7q3d | ||||||
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タイトル | Structure of the human CPLANE complex | ||||||
要素 |
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キーワード | PROTEIN TRANSPORT / ciliogenesis / ciliopathies / longin / lipid binding | ||||||
機能・相同性 | 機能・相同性情報 negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / axonemal basal plate / respiratory system development / tongue morphogenesis / auditory receptor cell morphogenesis / regulation of embryonic cell shape / septin cytoskeleton organization / digestive system development / embryonic body morphogenesis ...negative regulation of neural crest formation / negative regulation of fibroblast growth factor receptor signaling pathway involved in neural plate anterior/posterior pattern formation / axonemal basal plate / respiratory system development / tongue morphogenesis / auditory receptor cell morphogenesis / regulation of embryonic cell shape / septin cytoskeleton organization / digestive system development / embryonic body morphogenesis / regulation of ruffle assembly / regulation of fibroblast migration / podocyte cell migration / establishment of planar polarity / spinal cord dorsal/ventral patterning / motile cilium assembly / embryonic skeletal system morphogenesis / circulatory system development / cilium organization / negative regulation of cell division / positive regulation of cilium assembly / non-motile cilium assembly / regulation of smoothened signaling pathway / camera-type eye development / positive regulation of smoothened signaling pathway / motile cilium / regulation of establishment of cell polarity / limb development / neural tube development / hair follicle development / regulation of focal adhesion assembly / embryonic digit morphogenesis / hair follicle morphogenesis / smoothened signaling pathway / roof of mouth development / axoneme / cilium assembly / regulation of ossification / negative regulation of keratinocyte proliferation / Hedgehog 'off' state / keratinocyte differentiation / vesicle-mediated transport / ciliary basal body / negative regulation of cell migration / kidney development / neural tube closure / cell projection / establishment of protein localization / negative regulation of canonical Wnt signaling pathway / cilium / regulation of protein localization / protein transport / nervous system development / cytoskeleton / negative regulation of cell population proliferation / cell surface / extracellular exosome / plasma membrane / cytoplasm 類似検索 - 分子機能 | ||||||
生物種 | Homo sapiens (ヒト) | ||||||
手法 | 電子顕微鏡法 / 単粒子再構成法 / クライオ電子顕微鏡法 / 解像度: 3.35 Å | ||||||
データ登録者 | Langousis, G. / Cavadini, S. / Kempf, G. / Matthias, P. | ||||||
資金援助 | 1件
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引用 | ジャーナル: Sci Adv / 年: 2022 タイトル: Structure of the ciliogenesis-associated CPLANE complex. 著者: Gerasimos Langousis / Simone Cavadini / Niels Boegholm / Esben Lorentzen / Georg Kempf / Patrick Matthias / 要旨: Dysfunctional cilia cause pleiotropic human diseases termed ciliopathies. These hereditary maladies are often caused by defects in cilia assembly, a complex event that is regulated by the ...Dysfunctional cilia cause pleiotropic human diseases termed ciliopathies. These hereditary maladies are often caused by defects in cilia assembly, a complex event that is regulated by the ciliogenesis and planar polarity effector (CPLANE) proteins Wdpcp, Inturned, and Fuzzy. CPLANE proteins are essential for building the cilium and are mutated in multiple ciliopathies, yet their structure and molecular functions remain elusive. Here, we show that mammalian CPLANE proteins comprise a bona fide complex and report the near-atomic resolution structures of the human Wdpcp-Inturned-Fuzzy complex and of the mouse Wdpcp-Inturned-Fuzzy complex bound to the small guanosine triphosphatase Rsg1. Notably, the crescent-shaped CPLANE complex binds phospholipids such as phosphatidylinositol 3-phosphate via multiple modules and a CPLANE ciliopathy mutant exhibits aberrant lipid binding. Our study provides critical structural and functional insights into an enigmatic ciliogenesis-associated complex as well as unexpected molecular rationales for ciliopathies. | ||||||
履歴 |
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-構造の表示
構造ビューア | 分子: MolmilJmol/JSmol |
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-ダウンロードとリンク
-ダウンロード
PDBx/mmCIF形式 | 7q3d.cif.gz | 547.5 KB | 表示 | PDBx/mmCIF形式 |
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PDB形式 | pdb7q3d.ent.gz | 440.5 KB | 表示 | PDB形式 |
PDBx/mmJSON形式 | 7q3d.json.gz | ツリー表示 | PDBx/mmJSON形式 | |
その他 | その他のダウンロード |
-検証レポート
文書・要旨 | 7q3d_validation.pdf.gz | 1.2 MB | 表示 | wwPDB検証レポート |
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文書・詳細版 | 7q3d_full_validation.pdf.gz | 1.2 MB | 表示 | |
XML形式データ | 7q3d_validation.xml.gz | 46.6 KB | 表示 | |
CIF形式データ | 7q3d_validation.cif.gz | 70.3 KB | 表示 | |
アーカイブディレクトリ | https://data.pdbj.org/pub/pdb/validation_reports/q3/7q3d ftp://data.pdbj.org/pub/pdb/validation_reports/q3/7q3d | HTTPS FTP |
-関連構造データ
関連構造データ | 13789MC 7q3eC M: このデータのモデリングに利用したマップデータ C: 同じ文献を引用 (文献) |
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類似構造データ | 類似検索 - 機能・相同性F&H 検索 |
-リンク
-集合体
登録構造単位 |
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-要素
#1: タンパク質 | 分子量: 89649.320 Da / 分子数: 1 / 由来タイプ: 組換発現 / 由来: (組換発現) Homo sapiens (ヒト) / 遺伝子: WDPCP, BBS15, C2orf86, FRITZ 発現宿主: Spodoptera frugiperda (ツマジロクサヨトウ) 参照: UniProt: O95876 |
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#2: タンパク質 | 分子量: 108150.633 Da / 分子数: 1 / 由来タイプ: 組換発現 / 由来: (組換発現) Homo sapiens (ヒト) / 遺伝子: INTU, KIAA1284, PDZD6, PDZK6 発現宿主: Spodoptera frugiperda (ツマジロクサヨトウ) 参照: UniProt: Q9ULD6 |
#3: タンパク質 | 分子量: 45721.949 Da / 分子数: 1 / 由来タイプ: 組換発現 / 由来: (組換発現) Homo sapiens (ヒト) / 遺伝子: FUZ, FY 発現宿主: Spodoptera frugiperda (ツマジロクサヨトウ) 参照: UniProt: Q9BT04 |
-実験情報
-実験
実験 | 手法: 電子顕微鏡法 |
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EM実験 | 試料の集合状態: PARTICLE / 3次元再構成法: 単粒子再構成法 |
-試料調製
構成要素 | 名称: CPLANE / タイプ: COMPLEX / Entity ID: all / 由来: RECOMBINANT |
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由来(天然) | 生物種: Homo sapiens (ヒト) |
由来(組換発現) | 生物種: Spodoptera frugiperda (ツマジロクサヨトウ) |
緩衝液 | pH: 8 |
試料 | 包埋: NO / シャドウイング: NO / 染色: NO / 凍結: YES |
急速凍結 | 凍結剤: ETHANE |
-電子顕微鏡撮影
実験機器 | モデル: Titan Krios / 画像提供: FEI Company |
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顕微鏡 | モデル: FEI TITAN KRIOS |
電子銃 | 電子線源: FIELD EMISSION GUN / 加速電圧: 300 kV / 照射モード: SPOT SCAN |
電子レンズ | モード: BRIGHT FIELD |
撮影 | 電子線照射量: 1.04 e/Å2 フィルム・検出器のモデル: FEI FALCON IV (4k x 4k) |
-解析
EMソフトウェア |
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CTF補正 | タイプ: PHASE FLIPPING AND AMPLITUDE CORRECTION | |||||||||||||||||||||
3次元再構成 | 解像度: 3.35 Å / 解像度の算出法: FSC 0.143 CUT-OFF / 粒子像の数: 129902 / 対称性のタイプ: POINT | |||||||||||||||||||||
原子モデル構築 | プロトコル: FLEXIBLE FIT / 空間: REAL |