血影蛋白结构与功能研究进展

Progress in Structural and Functional Study of Spectrin

血影蛋白是一种细胞膜骨架蛋白,一个较大的异源二聚体蛋白,由亚基和亚基组成,并且具有典型的106个氨基酸构成的被称作血影蛋白重复体(repeat)的连续基序,在维持细胞膜的稳定、结构及形状中起重要作用.随着对血影蛋白结构与功能地深入研究,人们发现血影蛋白还参与多种生物途径,如细胞黏附及细胞伸展、细胞周期等.血影蛋白突变还会导致多种人类疾病,如遗传性溶血性贫血、5型脊髓小脑共济失调、癌症等.本研究主要从结构、功能及与疾病之间的关系这3个方面对血影蛋白的研究进展进行了详细阐述.

Spectrin is a cytoskeletal protein, a large heterodimeric protein which is composed of ct and 13 subunit with a modular structure made up of typically 106 amino acid contiguous motifs called ＇spectrin repeats＇, and plays an important role in maintaining the stability of the cell membrane, structure, and shape. In-depth analysis on spectrin structure and function has demonstrated that spectrin is involved in a variety of biological pathways, such as cell adhesion, cell spreading, and cell cycle. It was also found that spectrin mutations also lead to a variety of human diseases （hereditary hemolytic anemia, type 5 spinocerebellar ataxia, cancer, and so on）. This review focuses on the recent advances in structure and function of spectrin and the relationship between spectrin and disease.