• Ligation of integrin α2β1 on collagen prevents flow-induced NF-κB activation through a p38-dependent pathway that is activated locally at adhesion sites. (rupress.org)
  • We further found that the pericytic laminin isoforms differentially regulate mouse β-cells. (nature.com)
  • Immunostaining and confocal fluorescence microscopy show that the ECM proteins fibronectin, collagen I, and laminin are deposited throughout the entire 3D structure. (nature.com)
  • TGF-β1 is a multifunctional cytokine that has been shown to promote fibroblast proliferation and drive the stable accumulation of ECM proteins particularly collagen and fibronectin in vivo 14 , 15 . (nature.com)
  • The functionality of the dermal-epidermal junction depends strongly on the activity of specific structural proteins, in particular various Collagen types, Laminins, and Integrins. (dsm.com)
  • Laminin proteins are made of three different subunits called alpha, beta, and gamma. (medlineplus.gov)
  • Laminins are found in an intricate lattice of proteins and other molecules that forms in the spaces between cells (the extracellular matrix). (medlineplus.gov)
  • The laminins attach (bind) to other proteins in the extracellular matrix and in the membrane of muscle cells, which helps maintain the stability of muscle fibers. (medlineplus.gov)
  • Most LAMA2 gene mutations that cause the severe, early-onset form of LAMA2 -related muscular dystrophy result in the absence of functional laminin alpha-2 subunit. (medlineplus.gov)
  • Mutations that cause the milder, later-onset form usually result in a reduction (deficiency) of functional laminin alpha-2 subunit. (medlineplus.gov)
  • Mutations in laminin α2-subunit (Lmα2, encoded by LAMA2 ) are linked to approximately 30% of congenital muscular dystrophy cases. (jci.org)
  • Laminin LN domain mutations linked to several of the Lmα2-deficient muscular dystrophies are predicted to compromise polymerization. (jci.org)
  • In cultured myotubes, we determined that αLNNd expression increased myotube surface accumulation of polymerization-deficient recombinant laminins, with retention of collagen IV, reiterating the basement membrane (BM) changes observed in vivo. (jci.org)
  • Deficiency or absence of the laminin alpha-2 subunit results in a corresponding lack of laminin 2 and laminin 4, reducing the strength and stability of muscle tissue and leading to the signs and symptoms of LAMA2 -related muscular dystrophy. (medlineplus.gov)
  • Mice with a homozygous mutation in Lama2 ( dy2J mice) express a nonpolymerizing form of laminin-211 (Lm211) and are a model for ambulatory-type Lmα2-deficient muscular dystrophy. (jci.org)
  • The data herein support the hypothesis that engineered expression of αLNNd can overcome polymerization deficits to increase laminin, stabilize BM structure, and substantially ameliorate muscular dystrophy. (jci.org)