登録情報 データベース : EMDB / ID : EMD-3914 構造の表示 ダウンロードとリンクタイトル Substrate processing state 26S proteasome (SPS1) マップデータin situ reconstruction of Rat proteasome in substrate processing states 1 詳細 試料複合体 : Substrate processing state 26S proteasome (SPS1) 詳細 キーワード UPS / Substrate processing state / Neuron degeneration / HYDROLASE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
nuclear proteasome complex / Cross-presentation of soluble exogenous antigens (endosomes) / Proteasome assembly / N-glycan trimming in the ER and Calnexin/Calreticulin cycle / Regulation of ornithine decarboxylase (ODC) / Metalloprotease DUBs / Oxygen-dependent proline hydroxylation of Hypoxia-inducible Factor Alpha / Autodegradation of Cdh1 by Cdh1:APC/C / SCF-beta-TrCP mediated degradation of Emi1 / APC/C:Cdc20 mediated degradation of Securin ... nuclear proteasome complex / Cross-presentation of soluble exogenous antigens (endosomes) / Proteasome assembly / N-glycan trimming in the ER and Calnexin/Calreticulin cycle / Regulation of ornithine decarboxylase (ODC) / Metalloprotease DUBs / Oxygen-dependent proline hydroxylation of Hypoxia-inducible Factor Alpha / Autodegradation of Cdh1 by Cdh1:APC/C / SCF-beta-TrCP mediated degradation of Emi1 / APC/C:Cdc20 mediated degradation of Securin / APC/C:Cdh1 mediated degradation of Cdc20 and other APC/C:Cdh1 targeted proteins in late mitosis/early G1 / Cdc20:Phospho-APC/C mediated degradation of Cyclin A / SCF(Skp2)-mediated degradation of p27/p21 / Autodegradation of the E3 ubiquitin ligase COP1 / Asymmetric localization of PCP proteins / Degradation of DVL / Hedgehog ligand biogenesis / Dectin-1 mediated noncanonical NF-kB signaling / Degradation of GLI1 by the proteasome / Hedgehog 'on' state / TNFR2 non-canonical NF-kB pathway / NIK-->noncanonical NF-kB signaling / Assembly of the pre-replicative complex / CDK-mediated phosphorylation and removal of Cdc6 / G2/M Checkpoints / Ubiquitin-Mediated Degradation of Phosphorylated Cdc25A / Ubiquitin-dependent degradation of Cyclin D / The role of GTSE1 in G2/M progression after G2 checkpoint / FBXL7 down-regulates AURKA during mitotic entry and in early mitosis / RUNX1 regulates transcription of genes involved in differentiation of HSCs / Regulation of RUNX3 expression and activity / GSK3B and BTRC:CUL1-mediated-degradation of NFE2L2 / GLI3 is processed to GLI3R by the proteasome / Activation of NF-kappaB in B cells / Degradation of beta-catenin by the destruction complex / Degradation of AXIN / UCH proteinases / Regulation of RAS by GAPs / Orc1 removal from chromatin / Neddylation / AUF1 (hnRNP D0) binds and destabilizes mRNA / MAPK6/MAPK4 signaling / Regulation of PTEN stability and activity / KEAP1-NFE2L2 pathway / Separation of Sister Chromatids / fluid transport / Antigen processing: Ubiquitination & Proteasome degradation / ABC-family proteins mediated transport / Ub-specific processing proteases / positive regulation of inclusion body assembly / thyrotropin-releasing hormone receptor binding / host-mediated perturbation of viral transcription / cytosolic proteasome complex / proteasome accessory complex / integrator complex / meiosis I / proteasome regulatory particle / proteasome-activating activity / proteasome regulatory particle, lid subcomplex / proteasome regulatory particle, base subcomplex / metal-dependent deubiquitinase activity / protein K63-linked deubiquitination / Neutrophil degranulation / proteasome core complex / proteasome binding / regulation of protein catabolic process / myofibril / proteasome storage granule / general transcription initiation factor binding / blastocyst development / immune system process / endopeptidase activator activity / NF-kappaB binding / proteasome endopeptidase complex / proteasome core complex, beta-subunit complex / proteasome assembly / threonine-type endopeptidase activity / proteasome core complex, alpha-subunit complex / mRNA export from nucleus / inclusion body / enzyme regulator activity / regulation of proteasomal protein catabolic process / TBP-class protein binding / proteasome complex / proteolysis involved in protein catabolic process / sarcomere / stem cell differentiation / lipopolysaccharide binding / negative regulation of inflammatory response to antigenic stimulus / P-body / double-strand break repair via homologous recombination / : / response to virus / double-strand break repair via nonhomologous end joining / nuclear matrix / metallopeptidase activity / positive regulation of NF-kappaB transcription factor activity / peptidase activity / ubiquitin-dependent protein catabolic process / cytoplasmic vesicle 類似検索 - 分子機能 : / Ubiquitin interaction motif / : / 26S proteasome regulatory subunit RPN7/PSMD6 C-terminal helix / 26S proteasome non-ATPase regulatory subunit Rpn12 / 26S proteasome regulatory subunit, C-terminal / Proteasome regulatory subunit C-terminal / 26S proteasome regulatory subunit RPN5, C-terminal domain / : / 26S proteasome regulatory subunit RPN5 C-terminal domain ... : / Ubiquitin interaction motif / : / 26S proteasome regulatory subunit RPN7/PSMD6 C-terminal helix / 26S proteasome non-ATPase regulatory subunit Rpn12 / 26S proteasome regulatory subunit, C-terminal / Proteasome regulatory subunit C-terminal / 26S proteasome regulatory subunit RPN5, C-terminal domain / : / 26S proteasome regulatory subunit RPN5 C-terminal domain / PSD13 N-terminal repeats / 26S proteasome regulatory subunit Rpn6, N-terminal / 6S proteasome subunit Rpn6, C-terminal helix domain / 26S proteasome regulatory subunit RPN6 N-terminal domain / 26S proteasome subunit RPN6 C-terminal helix domain / 26S Proteasome non-ATPase regulatory subunit 13 / : / 26S proteasome subunit RPN2, N-terminal domain / 26S proteasome regulatory complex, non-ATPase subcomplex, Rpn2/Psmd1 subunit / 26S proteasome regulatory subunit RPN2, C-terminal / 26S proteasome regulatory subunit RPN2 C-terminal domain / : / 26S proteasome regulatory subunit RPN11 C-terminal domain / Proteasome subunit Rpn10 / 26S Proteasome non-ATPase regulatory subunit 7/8 / DSS1/SEM1 / DSS1/SEM1 family / DSS1_SEM1 / 26S proteasome regulatory complex, non-ATPase subcomplex, Rpn1 subunit / RPN1, N-terminal / 26S proteasome non-ATPase regulatory subunit RPN1, C-terminal / RPN1 N-terminal domain / 26S proteasome non-ATPase regulatory subunit RPN1 C-terminal / : / 26S proteasome regulatory subunit 7, OB domain / : / : / : / PSMD12/CSN4, N-terminal / 26S proteasome regulatory subunit Rpn7/COP9 signalosome complex subunit 1 / 26S proteasome regulatory subunit Rpn7, N-terminal / 26S proteasome subunit RPN7 / 26S Proteasome non-ATPase regulatory subunit 12/COP9 signalosome complex subunit 4 / Proteasome/cyclosome repeat / Proteasome/cyclosome repeat / Ubiquitin-interacting motif. / PCI/PINT associated module / : / von Willebrand factor type A domain / Proteasomal ATPase OB C-terminal domain / Proteasomal ATPase OB C-terminal domain / CSN8/PSMD8/EIF3K / CSN8/PSMD8/EIF3K family / Proteasome subunit alpha 1 / Rpn11/EIF3F, C-terminal / Maintenance of mitochondrial structure and function / HEAT repeats / : / motif in proteasome subunits, Int-6, Nip-1 and TRIP-15 / PCI domain / Proteasome component (PCI) domain / PCI domain profile. / Ubiquitin interacting motif / Ubiquitin-interacting motif (UIM) domain profile. / JAB1/Mov34/MPN/PAD-1 ubiquitin protease / Proteasome beta subunit, C-terminal / Proteasome beta subunits C terminal / Proteasome subunit beta 4 / Proteasome subunit beta 2 / Proteasome beta 3 subunit / Proteasome subunit alpha6 / Proteasome subunit alpha5 / Proteasome beta-type subunits signature. / Peptidase T1A, proteasome beta-subunit / Proteasome beta-type subunit, conserved site / Proteasome subunit A N-terminal signature / Proteasome alpha-type subunits signature. / Proteasome alpha-subunit, N-terminal domain / Proteasome subunit A N-terminal signature Add an annotation / von Willebrand factor (vWF) type A domain / VWFA domain profile. / Proteasome B-type subunit / Proteasome beta-type subunit profile. / : / Proteasome alpha-type subunit / Proteasome alpha-type subunit profile. / Proteasome subunit / Proteasome, subunit alpha/beta / von Willebrand factor, type A / AAA ATPase, AAA+ lid domain / AAA+ lid domain / JAB/MPN domain / JAB1/MPN/MOV34 metalloenzyme domain / ATPase, AAA-type, conserved site / AAA-protein family signature. / MPN domain / MPN domain profile. / Nucleophile aminohydrolases, N-terminal / von Willebrand factor A-like domain superfamily / ATPase family associated with various cellular activities (AAA) 類似検索 - ドメイン・相同性 26S proteasome non-ATPase regulatory subunit 13 / 26S proteasome complex subunit SEM1 / Proteasome 26S subunit, non-ATPase 7 / 26S proteasome non-ATPase regulatory subunit 8 / 26S proteasome non-ATPase regulatory subunit 11 / Proteasome 26S subunit, ATPase 6 / 26S proteasome non-ATPase regulatory subunit 1 / Proteasome subunit alpha type-2 / Proteasome subunit alpha type-1 / Proteasome subunit beta type-1 ... 26S proteasome non-ATPase regulatory subunit 13 / 26S proteasome complex subunit SEM1 / Proteasome 26S subunit, non-ATPase 7 / 26S proteasome non-ATPase regulatory subunit 8 / 26S proteasome non-ATPase regulatory subunit 11 / Proteasome 26S subunit, ATPase 6 / 26S proteasome non-ATPase regulatory subunit 1 / Proteasome subunit alpha type-2 / Proteasome subunit alpha type-1 / Proteasome subunit beta type-1 / Proteasome subunit alpha type-3 / Proteasome subunit alpha type-4 / Proteasome subunit beta type-6 / Proteasome subunit beta type-5 / Proteasome subunit alpha type-5 / Proteasome subunit beta type-4 / Proteasome subunit beta type-3 / Proteasome subunit beta type-2 / Proteasome subunit alpha type-7 / Proteasome subunit alpha type-6 / 26S proteasome regulatory subunit 4 / 26S proteasome regulatory subunit 8 / 26S proteasome non-ATPase regulatory subunit 2 / Proteasome (Prosome, macropain) 26S subunit, non-ATPase, 14 / Proteasome (Prosome, macropain) 26S subunit, non-ATPase, 3 / Proteasome (Prosome, macropain) 26S subunit, non-ATPase, 12 / 26S proteasome regulatory subunit 7 / 26S proteasome regulatory subunit 6A / 26S proteasome regulatory subunit 6B / Proteasome (Prosome, macropain) 26S subunit, ATPase 3 / 26S proteasome non-ATPase regulatory subunit 6 / 26S proteasome non-ATPase regulatory subunit 4 / Proteasome subunit beta type-7 類似検索 - 構成要素生物種 Rattus norvegicus (ドブネズミ)手法 サブトモグラム平均法 / クライオ電子顕微鏡法 / 解像度 : 15.4 Å 詳細 データ登録者Guo Q / Lehmer C 資金援助 ドイツ, 米国, 4件 詳細 詳細を隠すOrganization Grant number 国 European Commission FP7 GA ERC-2012-SyG_318987-ToPAG ドイツ European Commission FP7 GA ERC-2013-CoG_617198 DPR-MODELS ドイツ German Research Foundation SFB-1035/Project A01 ドイツ National Institutes of Health 9P41GM104601 米国
引用ジャーナル : Cell / 年 : 2018タイトル : In Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment.著者: Qiang Guo / Carina Lehmer / Antonio Martínez-Sánchez / Till Rudack / Florian Beck / Hannelore Hartmann / Manuela Pérez-Berlanga / Frédéric Frottin / Mark S Hipp / F Ulrich Hartl / Dieter ... 著者 : Qiang Guo / Carina Lehmer / Antonio Martínez-Sánchez / Till Rudack / Florian Beck / Hannelore Hartmann / Manuela Pérez-Berlanga / Frédéric Frottin / Mark S Hipp / F Ulrich Hartl / Dieter Edbauer / Wolfgang Baumeister / Rubén Fernández-Busnadiego / 要旨 : Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of many neurodegenerative diseases. Here, we address the elusive link between these phenomena by employing cryo- ... Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of many neurodegenerative diseases. Here, we address the elusive link between these phenomena by employing cryo-electron tomography to dissect the molecular architecture of protein aggregates within intact neurons at high resolution. We focus on the poly-Gly-Ala (poly-GA) aggregates resulting from aberrant translation of an expanded GGGGCC repeat in C9orf72, the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. We find that poly-GA aggregates consist of densely packed twisted ribbons that recruit numerous 26S proteasome complexes, while other macromolecules are largely excluded. Proximity to poly-GA ribbons stabilizes a transient substrate-processing conformation of the 26S proteasome, suggesting stalled degradation. Thus, poly-GA aggregates may compromise neuronal proteostasis by driving the accumulation and functional impairment of a large fraction of cellular proteasomes. 履歴 登録 2017年10月11日 - ヘッダ(付随情報) 公開 2017年12月20日 - マップ公開 2018年2月7日 - 更新 2024年5月15日 - 現状 2024年5月15日 処理サイト : PDBe / 状態 : 公開
すべて表示 表示を減らす