6PGW

Crystal structure of zebrafish Protocadherin-19 EC3-6

6PGW の概要

• エントリーDOI: 10.2210/pdb6pgw/pdb
• 分子名称: Protocadherin-19, CALCIUM ION, SODIUM ION, ... (4 entities in total)
• 機能のキーワード: protocadherin, pcdh19, epilepsy, calcium-binding, cell adhesion
• 由来する生物種: Danio rerio (Zebrafish)
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 49215.75

構造登録者

Cooper, S.R.,Hudson, J.D.,Sotomayor, M. (登録日: 2019-06-24, 公開日: 2020-12-30, 最終更新日: 2023-10-11)

主引用文献

Hudson, J.D.,Tamilselvan, E.,Sotomayor, M.,Cooper, S.R.
A complete Protocadherin-19 ectodomain model for evaluating epilepsy-causing mutations and potential protein interaction sites.
Structure, 29:1128-1143.e4, 2021

PubMed Abstract: Cadherin superfamily members play a critical role in differential adhesion during neurodevelopment, and their disruption has been linked to several neurodevelopmental disorders. Mutations in protocadherin-19 (PCDH19), a member of the δ-protocadherin subfamily of cadherins, cause a unique form of epilepsy called PCDH19 clustering epilepsy. While PCDH19 and other non-clustered δ-protocadherins form multimers with other members of the cadherin superfamily to alter adhesiveness, the specific protein surfaces responsible for these interactions are unknown. Only portions of the PCDH19 extracellular domain structure had been solved previously. Here, we present a structure of the missing segment from zebrafish Protocadherin-19 (Pcdh19) and create a complete ectodomain model. This model shows the structural environment for 97% of disease-causing missense mutations and reveals two potential surfaces for intermolecular interactions that could modify Pcdh19's adhesive strength and specificity.

PubMed: 34520737
DOI: 10.1016/j.str.2021.07.006

実験手法

X-RAY DIFFRACTION (3 Å)