登録情報 データベース : PDB / ID : 5kzw 構造の表示 ダウンロードとリンクタイトル Crystal structure of human GAA 要素Lysosomal alpha-glucosidase 詳細 キーワード HYDROLASE / Pompe disease / Glycogen storage disease 2 / human acid alpha glucosidase / glycoprotein / N-linked glycosylation / lysosomal enzyme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
vacuolar sequestering / autolysosome lumen / maltose metabolic process / alpha-1,4-glucosidase activity / sucrose metabolic process / Glycogen storage disease type II (GAA) / alpha-glucosidase activity / alpha-glucosidase / glycophagy / diaphragm contraction ... vacuolar sequestering / autolysosome lumen / maltose metabolic process / alpha-1,4-glucosidase activity / sucrose metabolic process / Glycogen storage disease type II (GAA) / alpha-glucosidase activity / alpha-glucosidase / glycophagy / diaphragm contraction / neuromuscular process controlling posture / : / tissue development / regulation of the force of heart contraction / glycogen catabolic process / aorta development / azurophil granule membrane / Glycogen breakdown (glycogenolysis) / muscle cell cellular homeostasis / lysosome organization / tertiary granule membrane / neuromuscular process controlling balance / ficolin-1-rich granule membrane / heart morphogenesis / cardiac muscle contraction / lysosomal lumen / locomotory behavior / glucose metabolic process / carbohydrate binding / lysosome / lysosomal membrane / intracellular membrane-bounded organelle / Neutrophil degranulation / extracellular exosome / membrane / plasma membrane 類似検索 - 分子機能 : / Spasmolytic Protein, domain 1 / Spasmolytic Protein; domain 1 / P-type trefoil, conserved site / P-type 'Trefoil' domain signature. / Trefoil (P-type) domain / P-type trefoil domain / P-type trefoil domain superfamily / P-type 'Trefoil' domain profile. / P or trefoil or TFF domain ... : / Spasmolytic Protein, domain 1 / Spasmolytic Protein; domain 1 / P-type trefoil, conserved site / P-type 'Trefoil' domain signature. / Trefoil (P-type) domain / P-type trefoil domain / P-type trefoil domain superfamily / P-type 'Trefoil' domain profile. / P or trefoil or TFF domain / Glycosyl hydrolases family 31, conserved site / Glycosyl hydrolases family 31 signature 2. / glycosyl hydrolase (family 31) / Glycosyl hydrolases family 31, active site / Glycosyl hydrolases family 31 active site. / Glycoside hydrolase family 31, N-terminal domain / Glycosyl hydrolase 31 N-terminal galactose mutarotase-like domain / : / Glycosyl hydrolase family 31 C-terminal domain / Glycoside hydrolase family 31 / Glycosyl hydrolases family 31 TIM-barrel domain / Galactose mutarotase-like domain superfamily / Golgi alpha-mannosidase II / Glycosyl hydrolase, all-beta / Few Secondary Structures / Irregular / Glycosidases / Glycoside hydrolase superfamily / TIM Barrel / Alpha-Beta Barrel / Immunoglobulin-like / Sandwich / Mainly Beta / Alpha Beta 類似検索 - ドメイン・相同性 beta-D-glucopyranose / alpha-D-glucopyranose / DI(HYDROXYETHYL)ETHER / TRIETHYLENE GLYCOL / Lysosomal alpha-glucosidase 類似検索 - 構成要素生物種 Homo sapiens (ヒト)手法 X線回折 / シンクロトロン / フーリエ合成 / 解像度 : 2 Å 詳細Model details Complex with DNJ データ登録者 Deming, D.T. / Garman, S.C. 資金援助 米国, 2件 詳細 詳細を隠す組織 認可番号 国 National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Disease (NIH/NIDDK) R01 DK76877 米国 National Institutes of Health/National Institute of General Medical Sciences (NIH/NIGMS) T32 GM008515 米国
引用ジャーナル : To be published タイトル : The structure of human GAA: structural basis of Pompe disease著者 : Deming, D.T. / Garman, S.C. 履歴 登録 2016年7月25日 登録サイト : RCSB / 処理サイト : RCSB改定 1.0 2017年7月26日 Provider : repository / タイプ : Initial release改定 1.1 2019年12月25日 Group : Author supporting evidence / Data collection / カテゴリ : chem_comp / pdbx_audit_supportItem : _chem_comp.type / _pdbx_audit_support.funding_organization改定 2.0 2020年7月29日 Group : Advisory / Atomic model ... Advisory / Atomic model / Data collection / Derived calculations / Refinement description / Structure summary カテゴリ : atom_site / chem_comp ... atom_site / chem_comp / entity / pdbx_branch_scheme / pdbx_chem_comp_identifier / pdbx_entity_branch / pdbx_entity_branch_descriptor / pdbx_entity_branch_link / pdbx_entity_branch_list / pdbx_entity_nonpoly / pdbx_nonpoly_scheme / pdbx_struct_assembly_gen / pdbx_unobs_or_zero_occ_atoms / refine / refine_hist / refine_ls_restr / refine_ls_shell / reflns / reflns_shell / struct_asym / struct_conn / struct_site / struct_site_gen Item : _atom_site.B_iso_or_equiv / _atom_site.Cartn_x ... _atom_site.B_iso_or_equiv / _atom_site.Cartn_x / _atom_site.Cartn_y / _atom_site.Cartn_z / _atom_site.auth_asym_id / _atom_site.auth_seq_id / _atom_site.label_alt_id / _atom_site.label_asym_id / _atom_site.label_entity_id / _chem_comp.name / _chem_comp.type / _pdbx_struct_assembly_gen.asym_id_list / _pdbx_unobs_or_zero_occ_atoms.label_asym_id / _refine.B_iso_max / _refine.B_iso_mean / _refine.B_iso_min / _refine.details / _refine.ls_R_factor_R_free / _refine.ls_R_factor_R_work / _refine.ls_R_factor_obs / _refine.ls_percent_reflns_obs / _refine_hist.cycle_id / _refine_hist.number_atoms_total / _refine_hist.pdbx_B_iso_mean_ligand / _refine_hist.pdbx_B_iso_mean_solvent / _refine_hist.pdbx_number_atoms_protein / _refine_hist.pdbx_number_residues_total / _refine_ls_shell.d_res_low / _refine_ls_shell.number_reflns_all / _reflns.pdbx_CC_half / _reflns_shell.pdbx_CC_half 解説 : Carbohydrate remediation / Provider : repository / タイプ : Remediation改定 2.1 2023年10月4日 Group : Data collection / Database references ... Data collection / Database references / Refinement description / Structure summary カテゴリ : chem_comp / chem_comp_atom ... chem_comp / chem_comp_atom / chem_comp_bond / database_2 / pdbx_initial_refinement_model Item : _chem_comp.pdbx_synonyms / _database_2.pdbx_DOI / _database_2.pdbx_database_accession
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