Barttin modulates trafficking and function of ClC-K channels
نویسندگان
چکیده
منابع مشابه
Barttin modulates trafficking and function of ClC-K channels.
Barttin is an accessory subunit of a subgroup of ClC-type chloride channels expressed in renal and inner ear epithelia. In this study, we examined the effects of barttin on two ClC-K channel isoforms, rat ClC-K1 and human ClC-Kb, using heterologous expression, patch clamping, confocal imaging, and flow cytometry. In the absence of barttin, only a small percentage of rClC-K1 and hClC-Kb channels...
متن کاملPhysiology and Pathophysiology of ClC-K/barttin Channels
ClC-K channels form a subgroup of anion channels within the ClC family of anion transport proteins. They are expressed predominantly in the kidney and in the inner ear, and are necessary for NaCl resorption in the loop of Henle and for K+ secretion by the stria vascularis. Subcellular distribution as well as the function of these channels are tightly regulated by an accessory subunit, barttin. ...
متن کاملModulation of ClC-K channel function by the accessory subunit barttin.
The human ClC-Ka/ClC-Kb and rodent ClC-K1/ClC-K2 channels are the pore-forming channel proteins participating in electrolyte transport across the thick (ClC-Kb, ClC-K2) and thin (ClC-Ka, ClC-K1) ascending limbs of Henle’s Loop; further nephron segments, such as distal convoluted tubule and collecting duct (ClC-K2, ClC-Kb); and the marginal cells of the stria vascularis in the inner ear (ClC-Ka,...
متن کاملDisease-causing dysfunctions of barttin in Bartter syndrome type IV.
Bartter syndrome type IV is an inherited human condition characterized by severe renal salt wasting and sensorineural deafness. The causal gene, BSND, encodes barttin, an accessory subunit of chloride channels located in the kidney and inner ear. Barttin modulates the stability, cell surface localization, and function of ClC-K channels; distinct mutations cause phenotypes of varying severity. F...
متن کاملReduced Membrane Insertion of CLC-K by V33L Barttin Results in Loss of Hearing, but Leaves Kidney Function Intact
In the mammalian ear, transduction of sound stimuli is initiated by K+ entry through mechano-sensitive channels into inner hair cells. K+ entry is driven by a positive endocochlear potential that is maintained by the marginal cell layer of the stria vascularis. This process requires basolateral K+ import by NKCC1 Na+-2Cl--K+ co-transporters as well as Cl- efflux through ClC-Ka/barttin or ClC-Kb...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2006
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.0601631103