• Dysfunctional structure of one particular laminin, laminin-211, is the cause of one form of congenital muscular dystrophy. (wikipedia.org)
  • Mutations of the LAMA2 gene are the most common cause of congenital muscular dystrophy that frequently leads to death in early childhood (Domogatskaya, 2012). (biolamina.com)
  • Laminins form independent networks and are associated with type IV collagen networks via entactin, fibronectin, and perlecan. (wikipedia.org)
  • For example, growth cones are repelled by netrin when grown on laminin-111 but are attracted to netrin when grown on fibronectin. (wikipedia.org)
  • MECs synthesize key extracellular matrix (ECM) proteins of the meninges such as various collagens, as well as fibronectin, laminin and tenascin, important for pia basal lamina integrity and thus for neuronal support and function [ 7 ]. (biomedcentral.com)
  • Laminins are heterotrimeric proteins with a high molecular mass (~400 to ~900 kDa) and possess three different chains (α, β and γ) encoded by five, four, and three paralogous genes in humans, respectively. (wikipedia.org)
  • The trimeric proteins intersect, composing a cruciform structure that is able to bind to other molecules of the extracellular matrix and cell membrane. (wikipedia.org)
  • Engineered constructs were compared to human SAS and assessed for specific cell-cell interaction markers as well as for extracellular matrix proteins found in human meninges. (biomedcentral.com)
  • They stained positively for specific cell-cell interaction markers indicative of a functional meningeal tissue, as well as extracellular matrix proteins found in human meninges. (biomedcentral.com)
  • Through these interactions, laminins critically contribute to cell attachment and differentiation, cell shape and movement, maintenance of tissue phenotype, and promotion of tissue survival. (wikipedia.org)
  • Laminin-111 is a major substrate along which nerve axons will grow, both in vivo and in vitro. (wikipedia.org)
  • For example, the peptide sequence [GTFALRGDNGDNGQ], which is located on the alpha-chain of laminin, promotes the adhesion of endothelial cells. (wikipedia.org)
  • citation needed] Laminins are enriched at the lesion site after peripheral nerve injury and are secreted by Schwann cells. (wikipedia.org)
  • defective laminins can cause muscles to form improperly, leading to a form of muscular dystrophy, lethal skin blistering disease (junctional epidermolysis bullosa), and/or defects of the kidney filter (nephrotic syndrome). (wikipedia.org)
  • In addition, many laminins had common names before either laminin nomenclature was in place. (wikipedia.org)
  • For a review of α2-laminin in skeletal muscle function, see Holmberg and Durbeej, 2012. (biolamina.com)
  • The structure of the laminin-G domain has been predicted to resemble that of pentraxin. (wikipedia.org)
  • Laminins are integral to the structural scaffolding of almost every tissue of an organism-secreted and incorporated into cell-associated extracellular matrices. (wikipedia.org)
  • Some of these biological functions of laminin have been associated with specific amino-acid sequences or fragments of laminin. (wikipedia.org)
  • The laminin stock solution is long-term stable when stored at -20°C to -80°C. Please refer to the product-specific CoA for shelf life details. (biolamina.com)
  • The presence of laminin-1 can influence how the growth cone responds to other cues. (wikipedia.org)
  • The major laminin of adult skeletal muscle is laminin-2 (also known as merosin), and only mutations of LAMA2 gene encoding laminin α2 cause muscular dystrophy. (medscape.com)
  • A large series of patients with LAMA2 mutations was described, highlighting the differences between the severe homogenous presentation typical of patients with absent merosinimmunostaining with the more heterogeneous presentation of those with residual merosin expression. (medscape.com)
  • MDC1A is caused by mutations in the laminin α2 gene ( LAMA2 ) linked to chromosome 6q22-q23 and inherited as autosomal recessive. (medscape.com)
  • Clinical variants of MDC1A occur with some mutations when only partial laminin-α2 deficiency is present. (medscape.com)
  • The Lama2(-/-) vascular endothelium had significant abnormalities, including altered integrity and composition of the endothelial basal lamina, inappropriate expression of embryonic vascular endothelial protein MECA32, substantially reduced pericyte coverage, and tight junction abnormalities. (nih.gov)
  • Laminin-211 appears to mediate these effects at least in part by dystroglycan receptor interactions, as preventing dystroglycan expression in neural cells led to a similar set of BBB abnormalities and gliovascular disturbances, which additionally included perturbed vascular endothelial glucose transporter-1 localization. (nih.gov)
  • For example, the peptide sequence [GTFALRGDNGDNGQ], which is located on the alpha-chain of laminin, promotes the adhesion of endothelial cells. (wikipedia.org)
  • Neurons of the peripheral nervous system express integrin receptors that attach to laminins and promote neuroregeneration after injury. (wikipedia.org)
  • Clues to the presence of laminin-α2 deficiency include MRI abnormalities, seizures, and demyelinating neuropathy. (medscape.com)
  • citation needed] This effect of laminin-111 probably occurs through a lowering of intracellular cyclic AMP. (wikipedia.org)
  • Through these interactions, laminins critically contribute to cell attachment and differentiation, cell shape and movement, maintenance of tissue phenotype, and promotion of tissue survival. (wikipedia.org)
  • These neurons are hence dependent upon RG extensions to support their migration from apical to basal regions. (frontiersin.org)
  • This review will focus on extracellular factors which influence RG morphology and pyramidal neuronal migration during normal development and their perturbations in pathology. (frontiersin.org)
  • Changes in one or several of these components influencing the RG extracellular environment can disrupt the development or maintenance of RG architecture on which neuronal migration relies, leading to a range of cortical malformations. (frontiersin.org)
  • Malfunctional laminin-521 in the kidney filter causes leakage of protein into the urine and nephrotic syndrome. (wikipedia.org)
  • In both rodent and primate, aRGs generate further basal intermediate neurogenic progenitors (IPs) residing in the subventricular zone (SVZ). (frontiersin.org)
  • Laminins are integral to the structural scaffolding of almost every tissue of an organism-secreted and incorporated into cell-associated extracellular matrices. (wikipedia.org)
  • Laminin-111 is a major substrate along which nerve axons will grow, both in vivo and in vitro. (wikipedia.org)
  • Each laminin is a heterodimer composed of a heavy chain (α) and two light chains (β and γ). (medscape.com)
  • Laminins are heterotrimeric proteins with a high molecular mass (~400 to ~900 kDa) and possess three different chains (α, β and γ) encoded by five, four, and three paralogous genes in humans, respectively. (wikipedia.org)
  • The laminins are combinations of different alpha-, beta-, and gamma-chains. (wikipedia.org)
  • Laminin-211 is composed of an α2, β1 and γ1 chains. (wikipedia.org)
  • White-matter changes are not correlated with the amount of laminin-α2, the patient's intelligence, or the presence of seizures. (medscape.com)
  • The presence of laminin-1 can influence how the growth cone responds to other cues. (wikipedia.org)
  • Fewer studies have identified the influence of the extracellular environment on this architecture. (frontiersin.org)
  • however, the role of laminins in BBB development remains unclear. (nih.gov)
  • During cortical development, RGs are present in different strategic positions: apical RGs (aRGs) have their cell bodies located in the ventricular zone with an apical process contacting the ventricle, while they also have a basal process extending radially to reach the pial surface of the cortex. (frontiersin.org)