Physiol Rev Information on EB 2010
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Physiol. Rev. 88: 639-672, 2008; doi:10.1152/physrev.00022.2007
0031-9333/08 $18.00
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Web of Science (28)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Hartzell, H. C.
Right arrow Articles by Chien, L.-T.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Hartzell, H. C.
Right arrow Articles by Chien, L.-T.

Molecular Physiology of Bestrophins: Multifunctional Membrane Proteins Linked to Best Disease and Other Retinopathies

H. Criss Hartzell, Zhiqiang Qu, Kuai Yu, Qinghuan Xiao and Li-Ting Chien

Department of Cell Biology, Center for Neurodegenerative Disease, Emory University School of Medicine, Atlanta, Georgia

This article reviews the current state of knowledge about the bestrophins, a newly identified family of proteins that can function both as Cl– channels and as regulators of voltage-gated Ca2+ channels. The founding member, human bestrophin-1 (hBest1), was identified as the gene responsible for a dominantly inherited, juvenile-onset form of macular degeneration called Best vitelliform macular dystrophy. Mutations in hBest1 have also been associated with a small fraction of adult-onset macular dystrophies. It is proposed that dysfunction of bestrophin results in abnormal fluid and ion transport by the retinal pigment epithelium, resulting in a weakened interface between the retinal pigment epithelium and photoreceptors. There is compelling evidence that bestrophins are Cl– channels, but bestrophins remain enigmatic because it is not clear that the Cl– channel function can explain Best disease. In addition to functioning as a Cl– channel, hBest1 also is able to regulate voltage-gated Ca2+ channels. Some bestrophins are activated by increases in intracellular Ca2+ concentration, but whether bestrophins are the molecular counterpart of Ca2+-activated Cl– channels remains in doubt. Bestrophins are also regulated by cell volume and may be a member of the volume-regulated anion channel family.




This article has been cited by other articles:


Home page
 J. Biol. Chem.Home page
J. Ousingsawat, J. R. Martins, R. Schreiber, J. R. Rock, B. D. Harfe, and K. Kunzelmann
Loss of TMEM16A Causes a Defect in Epithelial Ca2+-dependent Chloride Transport
J. Biol. Chem., October 16, 2009; 284(42): 28698 - 28703.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
H. Park, S.-J. Oh, K.-S. Han, D. H. Woo, H. Park, G. Mannaioni, S. F. Traynelis, and C. J. Lee
Bestrophin-1 Encodes for the Ca2+-Activated Anion Channel in Hippocampal Astrocytes
J. Neurosci., October 14, 2009; 29(41): 13063 - 13073.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
Q. Xiao, K. Yu, Y.-y. Cui, and H. C. Hartzell
Dysregulation of human bestrophin-1 by ceramide-induced dephosphorylation
J. Physiol., September 15, 2009; 587(18): 4379 - 4391.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
S. Pifferi, M. Dibattista, C. Sagheddu, A. Boccaccio, A. Al Qteishat, F. Ghirardi, R. Tirindelli, and A. Menini
Calcium-activated chloride currents in olfactory sensory neurons from mice lacking bestrophin-2
J. Physiol., September 1, 2009; 587(17): 4265 - 4279.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. Boudes, C. Sar, A. Menigoz, C. Hilaire, M. O. Pequignot, A. Kozlenkov, A. Marmorstein, P. Carroll, J. Valmier, and F. Scamps
Best1 Is a Gene Regulated by Nerve Injury and Required for Ca2+-Activated Cl- Current Expression in Axotomized Sensory Neurons
J. Neurosci., August 12, 2009; 29(32): 10063 - 10071.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
Z. Qu, W. Cheng, Y. Cui, Y. Cui, and J. Zheng
Human Disease-causing Mutations Disrupt an N-C-terminal Interaction and Channel Function of Bestrophin 1
J. Biol. Chem., June 12, 2009; 284(24): 16473 - 16481.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
H. C. Hartzell, K. Yu, Q. Xiao, L.-T. Chien, and Z. Qu
Anoctamin/TMEM16 family members are Ca2+-activated Cl\#8722; channels
J. Physiol., May 15, 2009; 587(10): 2127 - 2139.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
L. L. Marsey and J. P. Winpenny
Bestrophin expression and function in the human pancreatic duct cell line, CFPAC-1
J. Physiol., May 15, 2009; 587(10): 2211 - 2224.
[Abstract] [Full Text] [PDF]

Home page
 IOVSHome page
Y. Zhang, B. R. Davidson, W. D. Stamer, J. K. Barton, L. Y. Marmorstein, and A. D. Marmorstein
Enhanced Inflow and Outflow Rates Despite Lower IOP in Bestrophin-2-Deficient Mice
Invest. Ophthalmol. Vis. Sci., February 1, 2009; 50(2): 765 - 770.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
N. Esumi, S. Kachi, L. Hackler Jr, T. Masuda, Z. Yang, P. A. Campochiaro, and D. J. Zack
BEST1 expression in the retinal pigment epithelium is modulated by OTX family members
Hum. Mol. Genet., January 1, 2009; 18(1): 128 - 141.
[Abstract] [Full Text] [PDF]

Home page
 JGPHome page
R. A. Falin, R. Morrison, A.-J. L. Ham, and K. Strange
Identification of Regulatory Phosphorylation Sites in a Cell Volume- and Ste20 Kinase-dependent ClC Anion Channel
J. Gen. Physiol., December 29, 2008; 133(1): 29 - 42.
[Abstract] [Full Text] [PDF]

Home page
 JGPHome page
Q. Xiao, A. Prussia, K. Yu, Y.-y. Cui, and H. C. Hartzell
Regulation of Bestrophin Cl Channels by Calcium: Role of the C Terminus
J. Gen. Physiol., December 1, 2008; 132(6): 681 - 692.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Cell Physiol.Home page
K. E. O'Driscoll, W. J. Hatton, H. R Burkin, N. Leblanc, and F. C. Britton
Expression, localization, and functional properties of Bestrophin 3 channel isolated from mouse heart
Am J Physiol Cell Physiol, December 1, 2008; 295(6): C1610 - C1624.
[Abstract] [Full Text] [PDF]

Home page
 JGPHome page
L.-T. Chien and H. C. Hartzell
Rescue of Volume-regulated Anion Current by Bestrophin Mutants with Altered Charge Selectivity
J. Gen. Physiol., November 1, 2008; 132(5): 537 - 546.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
V. V. Matchkov, P. Larsen, E. V. Bouzinova, A. Rojek, D. M. B. Boedtkjer, V. Golubinskaya, F. S. Pedersen, C. Aalkjaer, and H. Nilsson
Bestrophin-3 (Vitelliform Macular Dystrophy 2-Like 3 Protein) Is Essential for the cGMP-Dependent Calcium-Activated Chloride Conductance in Vascular Smooth Muscle Cells
Circ. Res., October 10, 2008; 103(8): 864 - 872.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Cell Physiol.Home page
Z. Qu and H. C. Hartzell
Bestrophin Cl- channels are highly permeable to HCO3-
Am J Physiol Cell Physiol, June 1, 2008; 294(6): C1371 - C1377.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
K. Yu, Q. Xiao, G. Cui, A. Lee, and H. C. Hartzell
The Best Disease-Linked Cl- Channel hBest1 Regulates CaV1 (L-type) Ca2+ Channels via src-Homology-Binding Domains
J. Neurosci., May 28, 2008; 28(22): 5660 - 5670.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Visit Other APS Journals Online
Copyright © 2008 by the American Physiological Society.