The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/2500 - 1/5000. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Use a concentration of 1 - 4 µg/ml. using PFA/Triton X-100 Fixation/Permeabilization.
FunctionInvolved in stabilization of lens fiber cell cytoskeleton.
Involvement in diseaseDefects in BFSP2 are the cause of cataract autosomal dominant BFSP2-related (ADC-BFSP2) [MIM:611597]; also known as cataract autosomal dominant multiple types 1. Cataract is an opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function. Cataract autosomal dominant BFSP2-related is characterized by a variable phenotype that may or may not be consistent within a family. The opacities can be nuclear, sutural, stellate cortical, lamellar, cortical, nuclear embryonic, Y-sutural, punctate cortical, congenital or with juvenile- and adult-onset.
Sequence similaritiesBelongs to the intermediate filament family.
Cellular localizationMembrane. Cytoplasm. Cytoplasm > cytoskeleton. Membrane- and cytoskeleton-associated.