9HAN

Bovine collagen VI local refinement of C-terminal region

9HAN の概要

• エントリーDOI: 10.2210/pdb9han/pdb
• 関連するPDBエントリー: 9GTU
• EMDBエントリー: 51984
• 分子名称: Collagen type VI alpha 3 chain, Collagen type VI alpha 1 chain, Collagen type VI alpha 2 chain, ... (7 entities in total)
• 機能のキーワード: collagen, col6a1, col6a2, col6a3, triple-helix, bovine, microfibril, ecm, extracellular, matrix, fibril, structural protein
• 由来する生物種: Bos taurus (domestic cattle); 詳細
• タンパク質・核酸の鎖数: 6
• 化学式量合計: 1101125.06

構造登録者

Godwin, A.,Snee, M.,Roseman, A.,Baldock, C. (登録日: 2024-11-04, 公開日: 2025-08-20, 最終更新日: 2025-08-27)

主引用文献

Godwin, A.R.F.,Becker, M.H.,Dajani, R.,Snee, M.,Roseman, A.M.,Baldock, C.
Collagen VI microfibril structure reveals mechanism for molecular assembly and clustering of inherited pathogenic mutations.
Nat Commun, 16:7549-7549, 2025

PubMed Abstract: Collagen VI links the cell surface to the extracellular matrix to provide mechanical strength to most mammalian tissues, and is linked to human diseases including muscular dystrophy, fibrosis, cardiovascular disease and osteoarthritis. Collagen VI assembles from heterotrimers of three different α-chains into microfibrils, but there are many gaps in our knowledge of the molecular assembly process. Here, we determine the structures of both heterotrimeric mini-collagen VI constructs and collagen VI microfibrils, from mammalian tissue, using cryogenic-electron microscopy. These structures reveal a cysteine-rich coiled coil region involved in trimerisation as well as microfibril assembly. Furthermore, our structures show that pathogenic mutations are located at interaction sites involved in different steps of collagen VI assembly, from the trimeric-coiled coil region that mediates heterotrimerisation, to clusters of mutations in the triple-helical region involved in microfibril formation. Our microfibril structure provides a template for understanding supramolecular assembly, and offers a platform for rationale design of therapeutics for collagen VI pathologies.

PubMed: 40813585
DOI: 10.1038/s41467-025-62923-3

実験手法

ELECTRON MICROSCOPY (4.33 Å)