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BFSP1 Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a full length recombinant BFSP1.
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB |
|---|---|
| Primary Accession | Q12934 |
| Other Accession | BC041483 |
| Reactivity | Human |
| Host | mouse |
| Clonality | Monoclonal |
| Isotype | IgG |
| Clone Names | 1B11 |
| Calculated MW | 74544 Da |
| Gene ID | 631 |
|---|---|
| Other Names | Filensin, Beaded filament structural protein 1, Lens fiber cell beaded-filament structural protein CP 115, CP115, Lens intermediate filament-like heavy, LIFL-H, BFSP1 |
| Target/Specificity | BFSP1 (AAH41483, 1 a.a. ~ 540 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. |
| Dilution | WB~~1:500~1000 |
| Format | Clear, colorless solution in phosphate buffered saline, pH 7.2 . |
| Storage | Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing. |
| Precautions | BFSP1 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures. |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
Autosomal recessive juvenile onset cataract associated with mutation in BFSP1. Ramachandran RD, et al. Hum Genet, 2007 May. PMID 17225135.Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Strausberg RL, et al. Proc Natl Acad Sci U S A, 2002 Dec 24. PMID 12477932.The DNA sequence and comparative analysis of human chromosome 20. Deloukas P, et al. Nature, 2001 Dec 20-27. PMID 11780052.An autosomal dominant posterior polar cataract locus maps to human chromosome 20p12-q12. Yamada K, et al. Eur J Hum Genet, 2000 Jul. PMID 10909854.Isolation of the human beaded-filament structural protein 1 gene (BFSP1) and assignment to chromosome 20p11.23-p12.1. Rendtorff ND, et al. Genomics, 1998 Oct 1. PMID 9787085.
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