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Mouse Anti-BFSP2 Recombinant Antibody (CBYY-0523) (CBMAB-0526-YY)


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Summary

Host Animal
Mouse
Specificity
Human, Rat
Clone
CBYY-0523
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Human recombinant protein fragment corresponding to amino acids 184-415 of human BFSP2 produced in E.coli.
Host Species
Mouse
Specificity
Human, Rat
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:2,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Beaded Filament Structural Protein 2
Introduction
More than 99% of the vertebrate ocular lens is comprised of terminally differentiated lens fiber cells. Two lens-specific intermediate filament-like proteins, the protein product of this gene (phakinin), and filensin, are expressed only after fiber cell differentiation has begun. Bovineth proteins are found in a structurally unique cytoskeletal element that is referred to as the beaded filament (BF). Mutations in this gene have been associated with juvenile-onset, progressive cataracts and Dowling-Meara epidermolysis bullosa simplex.
Entrez Gene ID
8419
UniProt ID
Q13515
Alternative Names
Beaded Filament Structural Protein 2; Beaded Filament Structural Protein 2, Phakinin; Lens Fiber Cell Beaded Filament Protein CP 47; Lens Fiber Cell Beaded Filament Protein CP 49; Lens Intermediate Filament-Like Light; 49 KDa Cytoskeletal Protein; LIFL-L; CP47;
Function
Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA (PubMed:28935373).
Plays a role in maintenance of retinal lens optical clarity (By similarity).
Biological Process
Cell maturation Source: Ensembl
Intermediate filament organization Source: UniProtKB
Lens fiber cell development Source: Ensembl
Response to stimulus Source: UniProtKB-KW
Visual perception Source: UniProtKB-KW
Cellular Location
Cytoskeleton; Cytoplasm; Cell membrane; Cell cortex. Expressed primarily at the plasma membrane in peripheral lens fiber cells, however also localizes to the cytoplasm in mature lens fiber cells.
Involvement in disease
Cataract 12, multiple types (CTRCT12): 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. The opacities can be nuclear, sutural, stellate cortical, lamellar, cortical, nuclear embryonic, Y-sutural, punctate cortical, congenital or with juvenile- and adult-onset.
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For research use only. Not intended for any clinical use.

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