BFSP2 Antibody

Code CSB-PA615697LA01HU
Size US$166
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Image
  • Western Blot
    Positive WB detected in: Hela whole cell lysate, MCF-7 whole cell lysate, HepG2 whole cell lysate
    All lanes: BFSP2 antibody at 3.8µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 46 kDa
    Observed band size: 46 kDa

  • IHC image of CSB-PA615697LA01HU diluted at 1:400 and staining in paraffin-embedded human eye tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • Immunofluorescence staining of HepG2 cells with CSB-PA615697LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) BFSP2 Polyclonal antibody
Uniprot No.
Target Names
BFSP2
Alternative Names
BFSP2Phakinin antibody; 49 kDa cytoskeletal protein antibody; Beaded filament structural protein 2 antibody; Lens fiber cell beaded filament protein CP 47 antibody; CP47 antibody; Lens fiber cell beaded filament protein CP 49 antibody; CP49 antibody; Lens intermediate filament-like light antibody; LIFL-L antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Phakinin protein (11-110AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The BFSP2 Antibody (Product code: CSB-PA615697LA01HU) is Non-conjugated. For BFSP2 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA615697LB01HU BFSP2 Antibody, HRP conjugated ELISA
FITC CSB-PA615697LC01HU BFSP2 Antibody, FITC conjugated
Biotin CSB-PA615697LD01HU BFSP2 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:200-1:500
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA. Plays a role in maintenance of retinal lens optical clarity.
Gene References into Functions
  1. The results suggest that the N-terminal domain of CRYAA is required during in vitro complex formation with filensin and phakinin. PMID: 28935373
  2. We report a novel mutation (p.G112E) in the BFSP2 gene, underscoring the physiological importance of the beaded filament protein and supporting its role in human cataract formation. PMID: 24654948
  3. Data show that BFSP2 and PITX3, hitherto known to cause eye defects only in a dominant fashion, can also present recessively. PMID: 21836522
  4. The Y-sutural opacity in the lens might be the typical and earliest sign for cataract caused by the BFSP2 mutation. In addition, a myopia susceptibility locus in this region, which might also be associated with the mutation in BFSP2. PMID: 15570218
  5. Progressive isolated sutural cataract associated with deletion mutation of BFSP2 gene in Chinese pedigree. Physiological importance of beaded filament protein. Role of BFSP2 in human cataract formation. PMID: 17200662
  6. Progressive phenotype has provided more evidence for the heterogeneity of congenital cataract caused by BFSP2 mutations and for the important role BFSP2 plays in cataract formation. PMID: 17982427
  7. This is the first reported case of a congenital lamellar cataract phenotype associated with the mutation of Arg339His (P.R339H) in BFSP2. PMID: 18958306

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Involvement in disease
Cataract 12, multiple types (CTRCT12)
Subcellular Location
Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
Protein Families
Intermediate filament family
Tissue Specificity
Lens.
Database Links

HGNC: 1041

OMIM: 603212

KEGG: hsa:8419

STRING: 9606.ENSP00000304987

UniGene: Hs.659862

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