Chloride channel CLIC-like protein 1 (CLCC1)

Chloride channel CLIC-like protein 1 (CLCC1) is an ER-localized intracellular chloride ion channel critical for maintaining chloride homeostasis and calcium balance within the endoplasmic reticulum[1][3][2]. CLCC1 participates in proper protein folding, regulation of the unfolded protein response, and inter-organelle communication at ER-mitochondria contact sites[1][2]. Loss or mutation of CLCC1 impairs ER ion homeostasis, leading to ER stress, misfolded protein accumulation, cell death, and diseases such as autosomal recessive retinitis pigmentosa (RP32) and neurodegeneration[1][2][3]. In the pancreas, CLCC1 is required for β-cell function and insulin production, implicating it in glucose metabolism disorders[4]. No approved drugs target CLCC1, but loss-of-function mutations or disruption of activity reveal its importance in diseases with neuronal, retinal, or endocrine (islet) pathologies[1][2][3][4].

Other names

Chloride channel CLIC like 1CLCC1KIAA0761MCLCERAC1ER anion channel 1Mid-1-related chloride channel protein 1retinitis pigmentosa 32 (autosomal recessive)RP32

No known drugs; mechanistically, antagonism would disrupt ER chloride flux, alter Ca²⁺ homeostasis, cause ER stress, and cell death