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	Nanoscale conformational dynamics of human propionyl-CoA carboxylase.
ジャーナル・号・ページ	Structure, Vol. 34, Issue 1, Page 62-75.e4, Year 2026
掲載日	2026年1月8日
著者	Huifang Yan / Fengyun Ni / Qinghua Wang / Jianpeng Ma /
PubMed 要旨	Propionyl-CoA carboxylase (PCC) is a biotin-dependent mitochondrial enzyme responsible for propionyl-CoA catabolism. Deficiencies in human PCC (hPCC) cause propionic acidemia, a severe metabolic ...Propionyl-CoA carboxylase (PCC) is a biotin-dependent mitochondrial enzyme responsible for propionyl-CoA catabolism. Deficiencies in human PCC (hPCC) cause propionic acidemia, a severe metabolic disorder driven by toxic metabolite accumulation. Despite its therapeutic relevance, the structural basis of hPCC's catalytic function remains unresolved. Here, we present high-resolution cryo-EM structures of hPCC in four distinct states, unliganded, ADP-, AMPPNP-, and ATP-bound/substrate-bound, capturing the full trajectory of the biotin carboxyl carrier protein (BCCP) domain as it translocates between active sites. Our results reinforce the crucial role of nucleotide-gated B-lid subdomain in synchronizing catalysis through coupling with BCCP movement. Structural and biochemical analysis of 10 disease-associated variants reveals how mutations disrupt key domain interfaces and dynamic motions required for activity. These new insights define the mechanistic principles governing hPCC functions, establish a structural framework for understanding PCC-related disorders, and lay the groundwork for future efforts to engineer functional replacements or modulators for metabolic therapy.
リンク	Structure / PubMed:41197621
手法	EM (単粒子)
解像度	2.48 - 3.41 Å
構造データ	60500 8zux EMDB-60500, PDB-8zux: Structure of G668R-hPCC 手法: EM (単粒子) / 解像度: 2.68 Å 60501 8zuy EMDB-60501, PDB-8zuy: Structure of M695D-hPCC 手法: EM (単粒子) / 解像度: 2.63 Å 60502 8zuz EMDB-60502, PDB-8zuz: Structure of G131R-hPCC BCCP-BC Conformation 手法: EM (単粒子) / 解像度: 2.73 Å 60503 8zv0 EMDB-60503, PDB-8zv0: Structure of G131R-hPCC BCCP-CT Conformation 手法: EM (単粒子) / 解像度: 2.6 Å 60504 8zv1 EMDB-60504, PDB-8zv1: Structure of R165Q-hPCC BCCP-BC Conformation 手法: EM (単粒子) / 解像度: 2.8 Å 60505 8zv2 EMDB-60505, PDB-8zv2: Structure of R165Q-hPCC BCCP-CT Conformation 手法: EM (単粒子) / 解像度: 2.94 Å 60506 8zv3 EMDB-60506, PDB-8zv3: Structure of G202A/G203A-hPCC BCCP-BC Conformation 手法: EM (単粒子) / 解像度: 2.77 Å 60507 8zv4 EMDB-60507, PDB-8zv4: Structure of G202A/G203A-hPCC BCCP-CT Conformation 手法: EM (単粒子) / 解像度: 2.93 Å 60508 8zv5 EMDB-60508, PDB-8zv5: Structure of D440Y-hPCC BCCP-BC Conformation 手法: EM (単粒子) / 解像度: 3.1 Å 60509 8zv6 EMDB-60509, PDB-8zv6: Structure of D440Y-hPCC BCCP-CT Conformation 手法: EM (単粒子) / 解像度: 2.9 Å 64151 9uh1 EMDB-64151, PDB-9uh1: BCCP-BC Conformation of apo-hPCC 手法: EM (単粒子) / 解像度: 3.41 Å 64152 9uh2 EMDB-64152, PDB-9uh2: BCCP-CT Conformation of apo-hPCC 手法: EM (単粒子) / 解像度: 2.91 Å 64155 9uh5 EMDB-64155, PDB-9uh5: BCCP-BC Conformation of ADP-bound hPCC 手法: EM (単粒子) / 解像度: 2.93 Å 64156 9uh6 EMDB-64156, PDB-9uh6: BCCP-CT Conformation of ADP-bound hPCC 手法: EM (単粒子) / 解像度: 3.1 Å 64158 9uh8 EMDB-64158, PDB-9uh8: BCCP-BC Conformation of AMPPNP-bound hPCC 手法: EM (単粒子) / 解像度: 2.98 Å 64161 9uhb EMDB-64161, PDB-9uhb: BCCP-CT Conformation of AMPPNP-bound hPCC 手法: EM (単粒子) / 解像度: 3.16 Å 64173 9uhr EMDB-64173, PDB-9uhr: BCCP-BC Conformation of ATP-bound hPCC 手法: EM (単粒子) / 解像度: 2.48 Å 64174 9uhs EMDB-64174, PDB-9uhs: Complete a6b6 of ATP-bound hPCC 手法: EM (単粒子) / 解像度: 2.76 Å 64180 9uhy EMDB-64180, PDB-9uhy: BCCP-CT Conformation of ATP-bound hPCC 手法: EM (単粒子) / 解像度: 2.93 Å
化合物	ChemComp-ATP: ADENOSINE-5'-TRIPHOSPHATE / ATP, エネルギー貯蔵分子YM ChemComp-MG: Unknown entry ChemComp-BCT: BICARBONATE ION ChemComp-1VU: propionyl Coenzyme A ChemComp-BTN: BIOTIN ChemComp-ADP: ADENOSINE-5'-DIPHOSPHATE / ADP, エネルギー貯蔵分子YM ChemComp-ANP: PHOSPHOAMINOPHOSPHONIC ACID-ADENYLATE ESTER / AMP-PNP, エネルギー貯蔵分子類似体*YM
由来	homo sapiens (ヒト)
キーワード	TRANSFERASE / BIOTIN / CARBOXYLASE