Beaded Filament Structural Protein 1 (BFSP1)
CP115; CP94; LIFL-H; Filensin; Lens fiber cell beaded-filament structural protein CP 115; Lens intermediate filament-like heavy
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 (or CP115), 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). Cataract is the most frequent cause of visual impairment and blindness worldwide. While congenital cataracts are less frequent than age related cataracts, if not treated promptly they can result in irreversible neural blindness. The frequency of non-syndromic congenital cataract is estimated to be 1-6 cases per 10'000 children with one additional case being diagnosed during childhood.
Organism species: Homo sapiens (Human)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Hu01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Hu01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Hu01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Hu01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Hu01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Mus musculus (Mouse)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Mu01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Mu01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Mu01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Mu01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Mu01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rattus norvegicus (Rat)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Ra01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Ra01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Ra01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Ra01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Ra01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Canis familiaris; Canine (Dog)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Ca01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Ca01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Ca01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Ca01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Ca01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Sus scrofa; Porcine (Pig)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Po01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Po01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Po01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Po01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Po01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Bos taurus; Bovine (Cattle)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Bo01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Bo01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Bo01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Bo01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Bo01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Oryctolagus cuniculus (Rabbit)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Rb01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Rb01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Rb01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Rb01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Rb01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rhesus monkey (Simian)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPU516Mo01 | Recombinant Beaded Filament Structural Protein 1 (BFSP1) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAU516Mo01 | Monoclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IP. |
| PAU516Mo01 | Polyclonal Antibody to Beaded Filament Structural Protein 1 (BFSP1) | WB; IHC; ICC; IF. | |
| Assay Kits | CLU516Mo01 | CLIA Kit for Beaded Filament Structural Protein 1 (BFSP1) | CLIA assay for Antigen Detection |
| ELU516Mo01 | ELISA Kit for Beaded Filament Structural Protein 1 (BFSP1) | Enzyme-linked immunosorbent assay for Antigen Detection |
Reference
"Primary sequence, secondary structure, gene structure, and assembly properties suggests that the lens-specific cytoskeletal protein filensin represents a novel class of intermediate filament protein."
Exp. Eye Res. 66:625-644(1998) [PubMed] [Europe PMC] [Abstract]
"Isolation of the human beaded-filament structural protein 1 gene (BFSP1) and assignment to chromosome 20p11.23-p12.1."
Genomics 53:114-116(1998) [PubMed] [Europe PMC] [Abstract]
"The DNA sequence and comparative analysis of human chromosome 20."
Nature 414:865-871(2001) [PubMed] [Europe PMC] [Abstract]
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
"Chromosomal locations of the genes for the beaded filament proteins CP 115 and CP 47."
Curr. Eye Res. 14:11-18(1995) [PubMed] [Europe PMC] [Abstract]
"An autosomal dominant posterior polar cataract locus maps to human chromosome 20p12-q12."
Eur. J. Hum. Genet. 8:535-539(2000) [PubMed] [Europe PMC] [Abstract]
"Autosomal recessive juvenile onset cataract associated with mutation in BFSP1."
Hum. Genet. 121:475-482(2007) [PubMed] [Europe PMC] [Abstract]