6KAJ

Crystal structure of FKRP in complex with Ba ion

6KAJ の概要

• エントリーDOI: 10.2210/pdb6kaj/pdb
• 分子名称: Fukutin-related protein, ZINC ION, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... (7 entities in total)
• 機能のキーワード: glycosyltranferase, phospho ribitoyl tranferase, transferase
• 由来する生物種: Homo sapiens (Human)
• タンパク質・核酸の鎖数: 4
• 化学式量合計: 204909.12

構造登録者

Kuwabara, N. (登録日: 2019-06-23, 公開日: 2020-01-29, 最終更新日: 2024-10-30)

主引用文献

Kuwabara, N.,Imae, R.,Manya, H.,Tanaka, T.,Mizuno, M.,Tsumoto, H.,Kanagawa, M.,Kobayashi, K.,Toda, T.,Senda, T.,Endo, T.,Kato, R.
Crystal structures of fukutin-related protein (FKRP), a ribitol-phosphate transferase related to muscular dystrophy.
Nat Commun, 11:303-303, 2020

PubMed Abstract: α-Dystroglycan (α-DG) is a highly-glycosylated surface membrane protein. Defects in the O-mannosyl glycan of α-DG cause dystroglycanopathy, a group of congenital muscular dystrophies. The core M3 O-mannosyl glycan contains tandem ribitol-phosphate (RboP), a characteristic feature first found in mammals. Fukutin and fukutin-related protein (FKRP), whose mutated genes underlie dystroglycanopathy, sequentially transfer RboP from cytidine diphosphate-ribitol (CDP-Rbo) to form a tandem RboP unit in the core M3 glycan. Here, we report a series of crystal structures of FKRP with and without donor (CDP-Rbo) and/or acceptor [RboP-(phospho-)core M3 peptide] substrates. FKRP has N-terminal stem and C-terminal catalytic domains, and forms a tetramer both in crystal and in solution. In the acceptor complex, the phosphate group of RboP is recognized by the catalytic domain of one subunit, and a phosphate group on O-mannose is recognized by the stem domain of another subunit. Structure-based functional studies confirmed that the dimeric structure is essential for FKRP enzymatic activity.

PubMed: 31949166
DOI: 10.1038/s41467-019-14220-z

実験手法

X-RAY DIFFRACTION (2.2249 Å)