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Mouse Anti-BFSP1 Recombinant Antibody (1B23) (CBMAB-A0752-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
1B23
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
BFSP1 (NP_001186, 567 a.a. ~ 664 a.a) partial recombinant protein with GST tag.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
Buffer
PBS
Preservative
None
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Beaded Filament Structural Protein 1
Introduction
More than 99% of the vertebrate ocular lens is comprised of terminally differentiated lens fiber cells. Two lens-specific intermediate filament-like proteins, CP49 (also known as phakinin) and the protein product of this gene, filensin, are expressed only after fiber cell differentiation has begun. Both proteins are found in a structurally unique cytoskeletal element that is referred to as the beaded filament (BF). Mutations in this gene are the cause of autosomal recessive cortical juvenile-onset cataract. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]
Entrez Gene ID
631
UniProt ID
Q12934
Alternative Names
CP115; CP94; FILENSIN; LIFL-H
Function
Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA (PubMed:28935373).
Involved in altering the calcium regulation of MIP water permeability (PubMed:30790544).
Biological Process
Cell maturation Source: Ensembl
Intermediate filament organization Source: UniProtKB
Lens fiber cell development Source: GO_Central
Cellular Location
Cytoskeleton; Cytoplasm; Cell membrane; Cell cortex
Involvement in disease
Cataract 33, multiple types (CTRCT33): 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. CTRCT33 has juvenile-onset and the opacities are restricted to the cortex of the lens, not involving the nucleus.
PTM
Proteolytically cleaved during lens cell fiber differentiation with increased fragmentation as fiber cell age increases.
Filensin C-terminal fragment: Myristoylated at Gly-434 following proteolytic cleavage at Asp-433.
Filensin N-terminal fragment: Acetylated at Ala-42 following proteolytic cleavage at Leu-41.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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