层粘连蛋白β2抗体
规格：1mg/1ml
英文名： Laminin Beta 2
别名： LAMB2; Laminin beta 2; Laminin subunit beta 2; LAMS; S laminin; LAMB2_HUMAN.
分子量： 192kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Laminin
交叉反应：Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, .
细胞定位：细胞膜 细胞外基质 分泌型蛋白
产层粘连蛋白β2抗体品介绍：background: 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层粘连蛋白β2抗体 (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 层粘连蛋白β2抗体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. Gene ID: 3913 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
层粘连蛋白β2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 神经生物学 细胞粘附分子 细胞骨架 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid
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