BFSP2 Antibody

Datasheet

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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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) BFSP2 Polyclonal antibody

Uniprot No.

Q13515

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.

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

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol

Troubleshooting and FAQs

Antibody 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.

Customer Reviews and Q&A

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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

The results suggest that the N-terminal domain of CRYAA is required during in vitro complex formation with filensin and phakinin. PMID: 28935373
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
Data show that BFSP2 and PITX3, hitherto known to cause eye defects only in a dominant fashion, can also present recessively. PMID: 21836522
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
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
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
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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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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