2026-03-19 東北大学
図1. ジスフェルリン欠損マウスの骨格筋（A）ではレーザーで細胞膜損傷を起こすと筋線維の外に添加した蛍光色素（ピンク：FM4-64）が筋線維内に流入し続けます。一方でCK2α（緑：CK2α-GFP）を過剰発現すると（B）、蛍光色素（ピンク：FM4-64）の流入が少なく、細胞膜修復が改善していることがわかります。
＜関連情報＞
- https://www.tohoku.ac.jp/japanese/2026/03/press20260319-01-CK2.html
- https://www.tohoku.ac.jp/japanese/newimg/pressimg/tohokuuniv-press20260319_web01_CK2.pdf
- https://faseb.onlinelibrary.wiley.com/doi/10.1096/fj.202500773RRR
新規ジスフェルリン結合キナーゼCK2αはジスフェルリン症における細胞膜修復を促進する A Novel Dysferlin-Binding Kinase CK2α Promotes Plasma Membrane Repair in Dysferlinopathy
Naoko Nakamura, Naoki Suzuki, Shin-ichiro Kanno, Rei Yamanaka, Hiroya Ono, Rumiko Izumi, Rui Muliang, Christian Borgo, Akiyuki Ohno, Ryuhei Harada, Saki Saito, Yukino Funayama …
The FASEB Journal Published: 13 March 2026
DOI:https://doi.org/10.1096/fj.202500773RRR
ABSTRACT
Dysferlinopathy is an adult-onset form of muscular dystrophy caused by mutations in the dysferlin gene and is inherited in an autosomal recessive manner. Dysferlin is primarily known for its role in plasma membrane repair. Although several proteins associated with dysferlin have been identified, many aspects of its signaling pathways and protein–protein interactions remain unclear. Here, we focused on the region between the third and fourth C2 domains, where frequent genetic mutations occur and functional domains are concentrated, and identified the protein kinase CK2α (formerly known as casein kinase 2) as a novel dysferlin-binding protein. CK2α was found to accumulate at membrane injury sites along with dysferlin in mouse skeletal muscle, and membrane repair was delayed in CK2α knockout cells. Furthermore, overexpression of CK2α in dysferlin-deficient mouse muscle led to improved membrane repair. Additionally, we revealed that CK2α plays a role in phosphorylating annexin A1, which is known to bind to dysferlin and is involved in plasma membrane repair. Our results indicated that CK2α controls membrane repair by participating in the phosphorylation of annexin A1. The molecular interplay among dysferlin, CK2α, and phosphorylated annexin A1 represents a novel therapeutic target for promoting membrane repair.
