4.7 Review

beta II spectrin (SPTBN1): biological function and clinical potential in cancer and other diseases

Journal

INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES
Volume 17, Issue 1, Pages 32-49

Publisher

IVYSPRING INT PUBL
DOI: 10.7150/ijbs.52375

Keywords

beta II Spectrin; proliferation; cancers; heart; development; TGF-beta

Funding

  1. National Natural Science Foundation of China [81870331, 31701208]
  2. Natural Science Foundation of Shandong Province [ZR2017MC067]
  3. Qingdao municipal science and technology bureau project [18-2-2-65-jch, 19-6-1-2-nsh]

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Beta II spectrin plays crucial roles in the development of various organs and regulates multiple cellular functions. Dysfunction of beta II spectrin is associated with diseases, highlighting the potential therapeutic targets for future research. Understanding the regulatory mechanism of beta II spectrin signal inactivation and recovery may offer hope for the treatment of related diseases.
beta II spectrin, the most common isoform of non-erythrocyte spectrin, is a cytoskeleton protein present in all nucleated cells. Interestingly, beta II spectrin is essential for the development of various organs such as nerve, epithelium, inner ear, liver and heart. The functions of beta II spectrin include not only establishing and maintaining the cell structure but also regulating a variety of cellular functions, such as cell apoptosis, cell adhesion, cell spreading and cell cycle regulation. Notably, beta II spectrin dysfunction is associated with embryonic lethality and the DNA damage response. More recently, the detection of altered beta II spectrin expression in tumors indicated that beta II spectrin might be involved in the development and progression of cancer. Its mutations and disorders could result in developmental disabilities and various diseases. The versatile roles of beta II spectrin in disease have been examined in an increasing number of studies; nonetheless, the exact mechanisms of beta II spectrin are still poorly understood. Thus, we summarize the structural features and biological roles of beta II spectrin and discuss its molecular mechanisms and functions in development, homeostasis, regeneration and differentiation. This review highlight the potential effects of beta II spectrin dysfunction in cancer and other diseases, outstanding questions for the future investigation of therapeutic targets. The investigation of the regulatory mechanism of beta II spectrin signal inactivation and recovery may bring hope for future therapy of related diseases.

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