Recombinant Human Laminin subunit beta-2 (LAMB2), partial

中文名稱：

人LAMB2重組蛋白
貨號：

CSB-YP012731HU
規格：
來源：

Yeast
其他：

中文名稱：

人LAMB2重組蛋白
貨號：

CSB-EP012731HU-B
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

人LAMB2重組蛋白
貨號：

CSB-BP012731HU
規格：
來源：

Baculovirus
其他：

中文名稱：

人LAMB2重組蛋白
貨號：

CSB-MP012731HU
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

LAMB2
Uniprot No.：

P55268
別名：

Lamb2; LAMB2_HUMAN; Laminin B1s chain; Laminin beta 2; laminin S; Laminin subunit beta 2 ; Laminin subunit beta-2; laminin; beta 2 (laminin S); Laminin-11 subunit beta; Laminin-14 subunit beta; Laminin-15 subunit beta; Laminin-3 subunit beta; Laminin-4 subunit beta; Laminin-7 subunit beta; Laminin-9 subunit beta; LAMS; NPHS5; S laminin; S-LAM beta; S-laminin subunit beta
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

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：
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
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相關疾病：

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

Secreted, extracellular space, extracellular matrix, basement membrane. Note=S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.
數據庫鏈接：

HGNC: 6487

OMIM: 150325

KEGG: hsa:3913

STRING: 9606.ENSP00000307156

UniGene: Hs.439726