人 PLS1 抗体对
01AB02525
Plastin 1 (PLS1) is a calcium-regulated actin-bundling protein that is highly expressed in the stereocilia of inner ear hair cells and intestinal epithelial cells. It contains tandem calponin homology domains that cross-link actin filaments into tight, organized bundles, which are essential for maintaining the diameter and structural integrity of stereocilia. Through this actin-remodeling activity, PLS1 supports the formation of the epithelial brush border and sensory hair-cell function. Pathogenic variants in PLS1 are the cause of autosomal dominant non-syndromic sensorineural hearing loss (DFNA76), a condition characterized by progressive high-frequency hearing impairment. Loss of PLS1 leads to morphological defects in stereocilia and elevated auditory thresholds, establishing it as a critical component for cochlear hair-cell stability and auditory perception.
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技术参数
- 靶点基因名
- PLS1
- 蛋白别名
- plastin 1; PLS1
- UniProt
- Q14651
- 物种
- Human
产品详情
- 靶点名称
- PLS1
- 蛋白名称
- plastin 1
- 蛋白别名
- Plastin-1; Intestine-specific plastin; I-plastin
- 靶点基因名
- PLS1
- UniProt
- Q14651
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Plastin 1 (PLS1) is a calcium-regulated actin-bundling protein that is highly expressed in the stereocilia of inner ear hair cells and intestinal epithelial cells. It contains tandem calponin homology domains that cross-link actin filaments into tight, organized bundles, which are essential for maintaining the diameter and structural integrity of stereocilia. Through this actin-remodeling activity, PLS1 supports the formation of the epithelial brush border and sensory hair-cell function. Pathogenic variants in PLS1 are the cause of autosomal dominant non-syndromic sensorineural hearing loss (DFNA76), a condition characterized by progressive high-frequency hearing impairment. Loss of PLS1 leads to morphological defects in stereocilia and elevated auditory thresholds, establishing it as a critical component for cochlear hair-cell stability and auditory perception.