潜在型TGF-β结合蛋白2抗体
规格：1mg/1ml
英文名： LTBP2/C14orf141
别名： C14orf141; Chromosome 14 open reading frame 141; Latent TGF beta binding protein 2; Latent transforming growth factor beta binding protein 2; Latent-transforming growth factor beta-binding protein 2;
分子量： 191kDa
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 LTBP2/C1
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Rabbit,
细胞定位：分泌型蛋白
潜在型TGF-β结合蛋白2抗体产品介绍：background: The protein encoded by this gene belongs to the family of latent transforming growth factor (TGF)-beta binding proteins (LTBP), which are extracellular matrix proteins with multi-domain structure. This protein is the largest member of the LTBP family possessing unique regions and with most similarity to the fibrillins. It has thus been suggested that it may have multiple functions: as a member of the TGF-beta latent complex, as a structural component of microfibrils, and a role in cell adhesion. [provided by RefSeq, Jul 2008] Function: May play an integral structural role in elastic-fiber architectural organization and/or assembly. Subcellular Location: Secreted. Localized in nuchal ligament and aorta to the fibrillin-containing, microfibrillar component of elastic fibers. Tissue Specificity: Expressed in lung, weakly expressed in heart, placenta, liver and skeletal muscle. Post-translational modifications: Contains hydroxylated asparagine residues. DISEASE: Defects in LTBP2 are the cause of primary congenital glaucoma type 3D (GLC3D) [MIM:613086]. An autosomal recessive form of primary congenital 潜在型TGF-β结合蛋白2抗体glaucoma (PCG). PCG is characterized by marked increase of intraocular pressure at birth or early childhood, large ocular globes (buphthalmos) and corneal edema. It results from developmental defects of the trabecular meshwork and anterior chamber angle of the eye that prevent adequate drainage of aqueous humor. Defects in LTBP2 are the cause of microspherophakia (MCSPH) [MIM:251750]. It is a rare disease characterized by smaller and more spherical lenses than normal bilaterally, an increased anteroposterior thickness of the lens and highly myopic eyes.潜在型TGF-β结合蛋白2抗体 Lens dislocation or subluxation may occur, leading to defective accommodation. Similarity: Belongs to the LTBP family. Contains 20 EGF-like domains. Contains 4 TB (TGF-beta binding) domains. Gene ID: 4053 Database links: Entrez Gene: 4053 Human Omim: 602091 Human SwissProt: Q14767 Human Unigene: 512776 Human Unigene: 597522 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
潜在型TGF-β结合蛋白2抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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：细胞生物 **学 信号转导 生长因子和** 结合蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid