BFSP2 Antibody

中文名稱：

BFSP2兔多克隆抗體
貨號：

CSB-PA615697LA01HU
規格：

￥440
圖片：
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).
其他：

產品詳情
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) BFSP2 Polyclonal antibody
Uniprot No.：

Q13515
基因名：

BFSP2
別名：

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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Phakinin protein (11-110AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，BFSP2 Antibody (CSB-PA615697LA01HU)，的標記方式是Non-conjugated。對于BFSP2 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA615697LB01HU	BFSP2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA615697LC01HU	BFSP2 Antibody, FITC conjugated
Biotin	CSB-PA615697LD01HU	BFSP2 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC, IF

推薦稀釋比：

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
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
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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相關疾病：

Cataract 12, multiple types (CTRCT12)
亞細胞定位：

Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
蛋白家族：

Intermediate filament family
組織特異性：

Lens.
數據庫鏈接：

HGNC: 1041

OMIM: 603212

KEGG: hsa:8419

STRING: 9606.ENSP00000304987

UniGene: Hs.659862

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