期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 23, 期 7, 页码 -出版社
MDPI
DOI: 10.3390/ijms23073552
关键词
non-syndromic deafness; Usher syndrome; hearing; integrin signaling; mechanoelectrical transduction
资金
- Italian Ministry of University and Research [201744NR8S]
CIB2 is a protein capable of binding calcium and magnesium ions, playing an important role in cochlear hair cells and being associated with non-syndromic deafness. Besides its involvement in auditory physiology, CIB2 has diverse functions in various processes.
Calcium- and integrin-binding protein 2 (CIB2) is a small EF-hand protein capable of binding Mg2+ and Ca2+ ions. While its biological function remains largely unclear, an increasing number of studies have shown that CIB2 is an essential component of the mechano-transduction machinery that operates in cochlear hair cells. Mutations in the gene encoding CIB2 have been associated with non-syndromic deafness. In addition to playing an important role in the physiology of hearing, CIB2 has been implicated in a multitude of very different processes, ranging from integrin signaling in platelets and skeletal muscle to autophagy, suggesting extensive functional plasticity. In this review, we summarize the current understanding of biochemical and biophysical properties of CIB2 and the biological roles that have been proposed for the protein in a variety of processes. We also highlight the many molecular aspects that remain unclarified and deserve further investigation.
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