Laminin β-2 Polyclonal Antibody, 100μg, (ATB-T2529)

Background: Laminins, a family of extracellular matrix glycoproteins, are the major noncollagenous constituent of basement membranes. They have been implicated in a wide variety of biological processes including cell adhesion, differentiation, migration, signaling, neurite outgrowth and metastasis. Laminins, composed of 3 non identical chains: laminin alpha, beta and gamma (formerly A, B1, and B2, respectively), form a cruciform structure consisting of 3 short arms, each formed by a different chain, and a long arm composed of all 3 chains. Each laminin chain is a multidomain protein encoded by a distinct gene. Several isoforms of each chain have been described. Different alpha, beta and gamma chain isomers combine to give rise to different heterotrimeric laminin isoforms which are designated by Arabic numerals in the order of their discovery, i.e. alpha1beta1gamma1 heterotrimer is laminin 1. The biological functions of the different chains and trimer molecules are largely unknown, but some of the chains have been shown to differ with respect to their tissue distribution, presumably reflecting diverse functions in vivo. This gene encodes the beta chain isoform laminin, beta 2. The beta 2 chain contains the 7 structural domains typical of beta chains of laminin, including the short alpha region. However, unlike beta 1 chain, beta 2 has a more restricted tissue distribution. It is enriched in the basement membrane of muscles at the neuromuscular junctions, kidney glomerulus and vascular smooth muscle. Transgenic mice in which the beta 2 chain gene was inactivated by homologous recombination, showed defects in the maturation of neuromuscular junctions and impairment of glomerular filtration. Alternative splicing involving a non consensus 5' splice site (gc) in the 5' UTR of this gene has been reported. It was suggested that inefficient splicing of this first intron, which does not change the protein sequence, results in a greater abundance of the unspliced form of the transcript than the spliced form. The full-length nature of the spliced transcript is not known. [provided by RefSeq, Aug 2011]Product datasheet: Overview Product Description Laminin β-2 Polyclonal Antibody, 100μg, (ATB-T2529) Image Species Reactivities Human,Mouse,Rat Immunogen Synthesized peptide derived from the N-terminal region of human Laminin β-2. Properties Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Instructions-20°C/1 year References: A novel mutation of LAMB2 in a multigenerational mennonite family reveals a new phenotypic variant of Pierson syndrome. Mohney BG, et al. Ophthalmology, 2011 Jun. PMID 21236492 Free PMC Article Mutations in the human laminin beta2 (LAMB2) gene and the associated phenotypic spectrum. Matejas V, et al. Hum Mutat, 2010 Sep. PMID 20556798 Free PMC Article Ophthalmological aspects of Pierson syndrome. Bredrup C, et al. Am J Ophthalmol, 2008 Oct. PMID 18672223 Analysis of genes encoding laminin beta2 and related proteins in patients with Galloway-Mowat syndrome. Dietrich A, et al. Pediatr Nephrol, 2008 Oct. PMID 18594871 A milder variant of Pierson syndrome. Kagan M, et al. Pediatr Nephrol, 2008 Feb. PMID 17943323