Calcium-dependent chloride conductance in epithelia: Is there a contribution by Bestrophin?

Karl Kunzelmann, Vladimir M. Milenkovic, Melanie Spitzner, Rene Barro, Rainer Schreiber

Research output: Contribution to journalReview article

61 Citations (Scopus)

Abstract

Although known for more than 20 years, the molecular identity of epithelial Ca2+-activated Cl- channels remains obscure. Previous candidate proteins did not hold initial promises, and thus, new hope is put into the recently identified family of bestrophin proteins, as they reflect many of the properties found for native channels. Mutations in the bestrophin gene cause an autosomal form of macular dystrophy of the retina. Bestrophin 1 is assumed to form the basolateral Ca2+-activated Cl- channel in the retinal pigment epithelium of the eye. Other data suggest that bestrophin is a regulator of voltage gated Ca2+ channels. Structural information on bestrophins is available and a Cl- selective filter has been localized to the second transmembrane domain of bestrophin. It is possible that bestrophins function as physiologically regulated Cl- channels only in association with additional subunits and auxiliary proteins. Little is known about expression of bestrophin in gland acinar cells, which show a pronounced Ca2+-activated Cl- secretion. In airways and intestinal epithelia, bestrophins could be particularly important in diseases such as cystic fibrosis and secretory diarrhea.

Original languageEnglish (US)
Pages (from-to)879-889
Number of pages11
JournalPflugers Archiv European Journal of Physiology
Volume454
Issue number6
DOIs
StatePublished - Sep 1 2007
Externally publishedYes

Fingerprint

Calcium Chloride
Chlorides
Pigment Epithelium of Eye
Epithelium
Calcium
Retinal Pigment Epithelium
Acinar Cells
Protein Subunits
Macular Degeneration
Intestinal Mucosa
Cystic Fibrosis
Retina
Diarrhea
Proteins
Retinal Pigments
Mutation
Genes
Electric potential

Keywords

  • Bestrophin
  • Ca-activated Cl channels
  • CaCC
  • Cl channel
  • CLCA
  • Epithelium

ASJC Scopus subject areas

  • Physiology
  • Clinical Biochemistry
  • Physiology (medical)

Cite this

Calcium-dependent chloride conductance in epithelia : Is there a contribution by Bestrophin? / Kunzelmann, Karl; Milenkovic, Vladimir M.; Spitzner, Melanie; Barro, Rene; Schreiber, Rainer.

In: Pflugers Archiv European Journal of Physiology, Vol. 454, No. 6, 01.09.2007, p. 879-889.

Research output: Contribution to journalReview article

Kunzelmann, Karl ; Milenkovic, Vladimir M. ; Spitzner, Melanie ; Barro, Rene ; Schreiber, Rainer. / Calcium-dependent chloride conductance in epithelia : Is there a contribution by Bestrophin?. In: Pflugers Archiv European Journal of Physiology. 2007 ; Vol. 454, No. 6. pp. 879-889.
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