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Mouse Anti-BFSP1 Recombinant Antibody (FIL-7B10) (CBMAB-AP631LY)


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Summary

Host Animal
Mouse
Specificity
Cattle, Human, Sheep
Clone
FIL-7B10
Antibody Isotype
IgG1
Application
IHC, WB

Basic Information

Immunogen
Human and bovine lens filament enriched fraction.
Host Species
Mouse
Specificity
Cattle, Human, Sheep
Antibody Isotype
IgG1
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IHC1:400

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

Format
Ascites
Preservative
15mM sodium azide
Concentration
Batch dependent
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
This gene encodes a lens-specific intermediate filament-like protein named filensin. The encoded protein is expressed in lens fiber cells after differentiation has begun. This protein functions as a component of the beaded filament which is a cytoskeletal structure found in lens fiber cells. Mutations in this gene are the cause of autosomal recessive cortical juvenile-onset cataract. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]
Entrez Gene ID
Human631
Cattle281644
UniProt ID
HumanQ12934
CattleQ06002
Alternative Names
Beaded Filament Structural Protein 1; Lens Fiber Cell Beaded-Filament Structural Protein CP 115; Beaded Filament Structural Protein 1, Filensin; Lens Intermediate Filament-Like Heavy; LIFL-H; CP115;
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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