Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption

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Item Type:	Article
Title:	Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption
Creators Name:	Boesl, M.R., Stein, V., Huebner, C.A., Zdebik, A.A., Jordt, S.E., Mukhopadhyay, A.K., Davidoff, M.S., Holstein, A.F. and Jentsch, T.J.
Abstract:	The functions of some CLC Cl(-) channels are evident from human diseases that result from their mutations, but the role of the broadly expressed ClC-2 Cl(-) channel is less clear. Several important functions have been attributed to ClC-2, but contrary to these expectations ClC-2-deficient mice lacked overt abnormalities except for a severe degeneration of the retina and the testes, which led to selective male infertility. Seminiferous tubules did not develop lumina and germ cells failed to complete meiosis. Beginning around puberty there was a massive death of primary spermatocytes and later also of spermatogonia. Tubules were filled with abnormal Sertoli cells, which normally express ClC-2 in patches adjacent to germ cells. In the retina, photoreceptors lacked normal outer segments and degenerated between days P10 and P30. The current across the retinal pigment epithelium was severely reduced at P36. Thus, ClC-2 disruption entails the death of two cell types which depend on supporting cells that form the blood-testes and blood-retina barriers. We propose that ClC-2 is crucial for controlling the ionic environment of these cells.
Keywords:	Anion Channel, Epilepsy, Knock-Out, Retinal Degeneration, Sertoli Cell Only Syndrome, Animals, Mice
Source:	EMBO Journal
ISSN:	0261-4189
Publisher:	Oxford University Press
Volume:	20
Number:	6
Page Range:	1289-1299
Date:	15 March 2001
Official Publication:	https://doi.org/10.1093/emboj/20.6.1289
PubMed:	View item in PubMed

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