LAMB2 Antibody

說明書

中文名稱：

LAMB2兔多克隆抗體
貨號：

CSB-PA958054
規(guī)格：

￥2024
圖片：
Western blot analysis of extracts from RAW264.7 cells, using LAMB2 antibody.
其他：

產(chǎn)品詳情
產(chǎn)品評價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) LAMB2 Polyclonal antibody
Uniprot No.：

P55268
基因名：

LAMB2
宿主：

Rabbit
反應(yīng)種屬：

Human,Mouse,Rat
免疫原：

Synthesized peptide derived from internal of Human LAMB2.
免疫原種屬：

Homo sapiens (Human)
克隆類型：

Polyclonal
純化方式：

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度：

It differs from different batches. Please contact us to confirm it.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA,WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:3000

Protocols：

ELISA Protocol
Western Blotting (WB) 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(chǎn)品評價(jià)

靶點(diǎn)詳情

功能：

Binding to cells via a high affinity receptor, laminin is thought to mediate the attachment, migration and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components.
基因功能參考文獻(xiàn)：
1. In conclusion, we reported three Chinese cases with different LAMB2 mutations and different phenotypes, further broadening the range of eye and kidney pathology associated with mutations in LAMB2. PMID: 27925579
2. No pathogenic LAMB2 mutations were found in the cohort of children with steroid-resistant focal segmental glomerulosclerosis. PMID: 24856380
3. Comprehensive gene sequencing revealed a novel LAMB2 variant (c.440A --> G; His147R) that was homozygous in the 9 living, affected family members, observed at a frequency of 2.1% in the Old Order Mennonite population, and absent in 91 non-Mennonite controls PMID: 21236492
4. Novel mutations in steroid-resistant nephrotic syndrome diagnosed in Tunisian children were detected in LAMB2. PMID: 21125408
5. All previously reported and several novel LAMB2 mutations in relation to the associated phenotype in patients from 39 unrelated families, are reviewed. PMID: 20556798
6. Deficiency in LAMB2 causes congenital nephrosis with mesangial sclerosis and distinct eye abnormalities. PMID: 15367484
7. Hepalaminin, an autoantigen from chronic hepatitis C, consists of two domains of laminin beta-2 and a specific domain. PMID: 15603881
8. Mutations in the LAMB2 gene encoding laminin beta2, a component of the glomerular basement membrane and the neuro-muscular junction are responsible for the characteristic renal and eye abnormalities of Pierson syndrome. PMID: 16898484
9. Recessive missense mutations in LAMB2 expand the clinical spectrum of LAMB2-associated disorders. PMID: 16912710
10. LAMB2 has to be considered as culprit of milder disorders including nephrosis and variable ocular anomalies. PMID: 16921188
11. We demonstrated that overabundance of the beta2 chain of laminin is associated with increased basement membrane thickness and is possibly related to spermatogenic dysfunction PMID: 17804866
12. Milder phenotypes of Pearson Syndrome may be related to hypomorphic LAMB2 alleles. PMID: 17943323
13. Pierson syndrome is defined by the association of mental retardation, microcoria and DMS caused by mutation in LAMB2 gene PMID: 18065803
14. Study excluded LAMB2 as a candidate gene for Galloway-Mowat syndrome. PMID: 18594871
15. Loss-of-function mutations in laminin beta2 (LAMB2) cause a broad range of ocular pathology, emphasizing the importance of laminin beta2 in eye development. PMID: 18672223
顯示更多

收起更多
相關(guān)疾病：

Pierson syndrome (PIERSS); Nephrotic syndrome 5 with or without ocular abnormalities (NPHS5)
亞細(xì)胞定位：

Secreted, extracellular space, extracellular matrix, basement membrane. Note=S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.
數(shù)據(jù)庫鏈接：

HGNC: 6487

OMIM: 150325

KEGG: hsa:3913

STRING: 9606.ENSP00000307156

UniGene: Hs.439726

Most popular with customers

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
XPNPEP1 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
XDH Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WFDC2 Recombinant Monoclonal Antibody

Applications: ELISA, WB, FC

Species Reactivity: Human
WDR5 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human