Mindell J A, Maduke M
Howard Hughes Medical Institute and Department of Biochemistry, Brandeis University, Waltham, MA 02454, USA.
Genome Biol. 2001;2(2):REVIEWS3003. doi: 10.1186/gb-2001-2-2-reviews3003. Epub 2001 Feb 7.
Chloride-conducting ion channels of the ClC family are emerging as critical contributors to a host of biological processes. These polytopic membrane proteins form aqueous pathways through which anions are selectively allowed to pass down their concentration gradients. The ClCs are found in nearly all organisms, with members in every mammalian tissue, yet relatively little is known about their mechanism or regulation. It is clear, however, that they are fundamentally different in molecular construction and mechanism from the well-known potassium-, sodium-, and calcium-selective channels. The medical importance of ClC channels - four inherited diseases have been blamed on familial ClC dysfunction to date - highlights their diverse physiological functions and provides strong motivation for further study.
ClC家族的氯离子传导离子通道正逐渐成为众多生物过程的关键贡献者。这些多跨膜蛋白形成水性通道,阴离子可通过这些通道选择性地顺着其浓度梯度通过。ClC通道几乎存在于所有生物体中,在每个哺乳动物组织中都有成员,但对其机制或调节的了解相对较少。然而,很明显,它们在分子结构和机制上与著名的钾离子、钠离子和钙离子选择性通道有根本不同。ClC通道的医学重要性——迄今为止,已有四种遗传性疾病被归咎于家族性ClC功能障碍——凸显了它们多样的生理功能,并为进一步研究提供了强大动力。