Product Datasheet
BEST1 Antibody
Catalog Number: 33002
Technical:tech@swbio.com
Information:info@swbio.com
Description
- Swiss-Prot No.:
- Swiss-Prot:O76090
NCBI Gene ID:7439
- Form of Antibody:
- Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
- Immunogen:
- Recombinant protein of human BEST1.
- appl_detail:
- Western blotting: 1:500 - 1:2000
Immunohistochemistry: 1:50 - 1:200
- other_names:
- ARB; BMD; BEST; RP50; VMD2
- Purification:
- Antibodies were purified by affinity purification using immunogen.
- Specificity:
- The antibody detects endogenous level of total BEST1 protein.
- Background:
- This gene encodes a member of the bestrophin gene family. This small gene family is characterized by proteins with a highly conserved N-terminus with four to six transmembrane domains. Bestrophins may form chloride ion channels or may regulate voltage-gated L-type calcium-ion channels. Bestrophins are generally believed to form calcium-activated chloride-ion channels in epithelial cells but they have also been shown to be highly permeable to bicarbonate ion transport in retinal tissue. Mutations in this gene are responsible for juvenile-onset vitelliform macular dystrophy (VMD2), also known as Best macular dystrophy, in addition to adult-onset vitelliform macular dystrophy (AVMD) and other retinopathies. Alternative splicing results in multiple variants encoding distinct isoforms.
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