Bestrophins

A protein family characterized by a highly conserved N-terminus and four to six transmembrane helices; they function as bicarbonate permeable, calcium-activated chloride channels. Bestrophin-1 (BEST-1) and bestrophin-2 are highly expressed in human RETINAL PIGMENT EPITHELIUM cells and mutations in the BEST-1 gene are associated with VITELLIFORM MACULAR DYSTROPHY, TYPE 2.

Also Known As:

Networked: 100 relevant articles (2 outcomes, 3 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Hartzell, H Criss: 13 articles (03/2010 - 01/2005)
2.	Marmorstein, Alan D: 12 articles (01/2018 - 08/2003)
3.	Marmorstein, Lihua Y: 11 articles (01/2018 - 08/2003)
4.	Yu, Kuai: 8 articles (12/2013 - 11/2006)
5.	Stanton, J Brett: 7 articles (01/2015 - 10/2004)
6.	Weber, Bernhard H F: 6 articles (02/2020 - 01/2007)
7.	Johnson, Adiv A: 6 articles (01/2018 - 12/2013)
8.	Chien, Li-Ting: 6 articles (11/2008 - 10/2005)
9.	Black, Graeme C M: 5 articles (06/2011 - 10/2004)
10.	Manson, Forbes D C: 5 articles (06/2011 - 10/2004)

Related Diseases

1.	Inflammation (Inflammations) 01/01/2014 - "Bestrophin 3 ameliorates TNFα-induced inflammation by inhibiting NF-κB activation in endothelial cells." 01/01/2022 - "An inflammation response-related gene profile was generated using LASSO-Cox regression analysis of Homo sapiens bestrophin 1 (BEST1), Chemokine (C-C motif) ligand 2 (CCL2), and plasminogen activator, urokinase receptor (PLAUR). "
2.	Filariasis 04/17/2021 - "Therapeutic Efficacy of Anti-Bestrophin Antibodies against Experimental Filariasis: Immunological, Immune-Informatics and Immune Simulation Investigations."
3.	Vitelliform Macular Dystrophy 08/01/2015 - "The purpose of the current study was to investigate the 11 bestrophin-1 (BEST1) exons in patients with best vitelliform macular dystrophy (BVMD), and to characterize the associated clinical features. " 07/04/2022 - "Impaired Bestrophin Channel Activity in an iPSC-RPE Model of Best Vitelliform Macular Dystrophy (BVMD) from an Early Onset Patient Carrying the P77S Dominant Mutation." 01/01/2022 - "Best vitelliform macular dystrophy (BVMD) is a slowly progressive macular disease caused by a pathogenic variant of the Bestrophin (BEST1) gene. " 07/02/2019 - "The Y227N mutation affects bestrophin-1 protein stability and impairs sperm function in a mouse model of Best vitelliform macular dystrophy." 10/01/2017 - "Bestrophin 1 gene analysis and associated clinical findings in a Chinese patient with Best vitelliform macular dystrophy."
4.	Cystic Fibrosis (Mucoviscidosis) 05/15/2009 - "In the present study, the expression of bestrophins has been investigated in the cystic fibrosis pancreatic duct cell line, CFPAC-1. " 03/01/2005 - "All cardiac Cl- channels described to date may be encoded by five different Cl- channel genes: the PKA- and PKC-activated cystic fibrosis tansmembrane conductance regulator (CFTR), the volume-regulated ClC-2 and ClC-3, and the Ca2+-activated CLCA or Bestrophin. " 02/01/2010 - "Thus, bestrophin 1 regulates compartmentalized Ca(2+) signaling that plays an essential role in Best macular dystrophy, inflammatory diseases such as cystic fibrosis, as well as proliferation." 09/01/2007 - "In airways and intestinal epithelia, bestrophins could be particularly important in diseases such as cystic fibrosis and secretory diarrhea." 01/01/2006 - "On the basis of molecular biologic findings, ligand-gated Cl(-) channels in synapses, cystic fibrosis transmembrane conductors (CFTRs) and ClC channel types have been established, followed by bestrophin and possibly by tweety, which encode Ca(2+)-activated Cl(-) channels. "
5.	Pain (Aches) 07/01/2015 - "Previous studies suggest that CaCCs family members bestrophin-1 and anoctamin-1 are involved in inflammatory pain. "

Related Drugs and Biologics

1.	Antibodies
2.	Chloride Channels (Chloride Channel)
3.	Anoctamin-1
4.	Proteins (Proteins, Gene)
5.	Anions
6.	Bestrophins
7.	Retinaldehyde (Retinal)
8.	Retinal Pigments (Pigments, Visual)
9.	Membrane Proteins (Integral Membrane Proteins)
10.	Urokinase Plasminogen Activator Receptors

Related Therapies and Procedures

1.	Ligation
2.	Spinal Injections