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Laminin Beta 2 Rabbit pAb (bs-7504R)  
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產(chǎn)品編號(hào) bs-7504R
英文名稱 Laminin Beta 2 Rabbit pAb
中文名稱 層粘連蛋白β2抗體
別    名 LAMB2; Laminin beta 2; Laminin subunit beta 2; LAMS; S laminin; LAMB2_HUMAN.  
研究領(lǐng)域 心血管  神經(jīng)生物學(xué)  細(xì)胞粘附分子  細(xì)胞骨架  細(xì)胞外基質(zhì)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human (predicted: Mouse,Rat,Rabbit,Sheep,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 192 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞膜 細(xì)胞外基質(zhì) 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Laminin Beta 2/Laminin S: 1701-1798/1798 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 Laminin S binds 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. Laminin S is a complex glycoprotein, consisting of three different polypeptide chains (alpha, beta, gamma), which are bound to each other by disulfide bonds into a cross-shaped molecule comprising one long and three short arms with globules at each end. Beta 2 is a subunit of laminin 3 (Laminin S), laminin 4 (S merosin), and laminin 7 (KS laminin).

Function:
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.

Subunit:
Laminin is a complex glycoprotein, consisting of three different polypeptide chains (alpha, beta, gamma), which are bound to each other by disulfide bonds into a cross-shaped molecule comprising one long and three short arms with globules at each end. Beta-2 is a subunit of laminin-3 (laminin-121 or S-laminin), laminin-4 (laminin-221 or S-merosin), laminin-7 (laminin-321 or KS-laminin), laminin-9 (laminin-421), laminin-11 (laminin-521), laminin-14 (laminin-423) and laminin-15 (laminin-523).

Subcellular Location:
Secreted, extracellular space, extracellular matrix, basement membrane. Note=S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.

DISEASE:
Defects in LAMB2 are the cause of Pierson syndrome (PIERSS) [MIM:609049]; also known as microcoria-congenital nephrotic syndrome. Pierson syndrome is characterized by nephrotic syndrome with neonatal onset, diffuse mesangial sclerosis and eye abnormalities with microcoria as the leading clinical feature. Death usually occurs within the first weeks of life. Disease severity depends on the mutation type: nontruncating LAMB2 mutations may display variable phenotypes ranging from a milder variant of Pierson syndrome to isolated congenital nephrotic syndrome.
Defects in LAMB2 are the cause of nephrotic syndrome type 5 with or without ocular abnormalities (NPHS5) [MIM:614199]. NPHS5 is a renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. NPHS5 is characterized by very early onset of progressive renal failure. A subset of patients may develop mild ocular anomalies, such as myopia, nystagmus, and strabismus.

Similarity:
Contains 13 laminin EGF-like domains.
Contains 1 laminin IV type B domain.
Contains 1 laminin N-terminal domain.

SWISS:
Q16787

Gene ID:
3918

Database links:

Entrez Gene: 3913 Human

Entrez Gene: 3909 Human

Entrez Gene: 3914 Human

Entrez Gene: 3918 Human

SwissProt: P55268 Human

SwissProt: Q13751 Human

SwissProt: Q13753 Human

SwissProt: Q16787 Human

SwissProt: Q61087 Mouse

SwissProt: Q61092 Mouse

SwissProt: Q61789 Mouse



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; A
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