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  • zebrafish
  • In the zebrafish, the α1, β1, and γ1 chains that make up the laminin 1 complex are essential for normal embryonic development and have been shown to be particularly important in notochord morphogenesis and maintenance ( 10 , 11 ). (pnas.org)
  • Homozygous lama2 mutant zebrafish, designated lama2cl501/cl501, exhibited reduced motor function and progressive degeneration of skeletal muscles and died at 8-15 days post fertilization. (harvard.edu)
  • Collagen
  • Biochemistry of Collagens, Laminins, and Elastin: Structure, Function, and Biomarkers provides a comprehensive introduction to collagen and structural proteins. (schweitzer-online.de)
  • Upon binding, laminin interacts with dystroglycan, mobilizes utrophin, and assembles a `nascent' basement membrane, independent of integrin, that is completed by incorporation of type IV collagen. (biologists.org)
  • 1996
  • It is thought to link the LG modules of α1- and α2-laminins to the cytoskeleton through its interaction with cytoskeletal dystrophin and/or utrophin (reviewed by Henry and Campbell, 1996 ). (biologists.org)
  • glycoprotein
  • Together with the dystrophin-glycoprotein complex laminin-211 forms a link between the basement membrane and the intracellular cytoskeleton protecting the muscle fiber from contraction-induced damage ( Ervasti and Campbell, 1993 ). (frontiersin.org)
  • Most commonly, the proposed mechanism of cellular pathology centers on the structural role of LAMA2, through its interaction with the dystrophin-associated glycoprotein complex (DGC), necessary for maintenance of sarcolemmal integrity ( 15 ). (pnas.org)
  • molecules
  • In addition to their structural role, laminins also act as signaling molecules through receptors such as integrins ( 8 ). (pnas.org)
  • This review deals with biological and pathological aspects of various isoforms of the matrix molecules fibronectin and laminin. (springer.com)
  • phenotype
  • The classical phenotype is associated with complete LAMA2 deficiency and pathological symptoms of muscle degeneration, fibrosis, and white matter abnormalities within the CNS ( 5 ). (pnas.org)
  • dystroglycan
  • The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sacrolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization. (genetex.com)
  • LAMA2 is known to bind directly to α-dystroglycan, the component of the DGC most distal to the sarcolemma, and thereby to anchor the muscle cell membrane to the ECM ( 16 ). (pnas.org)
  • In this study we have investigated the contributions of dystroglycan,β 1-integrin, and corresponding interacting long arm globular LG modules of laminin to Schwann cell basement membrane formation in vitro. (biologists.org)
  • We found that dystroglycan and utrophin underwent a dynamic rearrangement with exogenous laminin into a dense (reticular) structure with classical ultrastructural features of a basement membrane. (biologists.org)
  • embryonic
  • The laminin chain constitution of basement membranes switches from embryonic or proliferatively active to adult terminally differentiated tissues [disappearance of the laminin β2 (s) chain] and depends on the tissue type. (springer.com)
  • chain
  • We demonstrate that expression of muscle-specific miR-1, miR-133a, and miR-206 is deregulated in laminin α2 chain-deficient muscle. (frontiersin.org)
  • Finally, we show that plasma levels of muscle-specific miRNAs are markedly elevated in laminin α2 chain-deficient mice and partially normalized in response to proteasome inhibition therapy. (frontiersin.org)
  • chromosome
  • Therefore, we have analyzed the status of chromosome 1q, TNC, LAMA2, and NELL2 expression in a series of pediatric PF ependymomas in terms of their frequency, associations among themselves, and clinical parameters, as well as their prognostic impact. (biomedcentral.com)
  • peripheral
  • Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. (genetex.com)
  • Also acts as a receptor for M.leprae in peripheral nerve Schwann cells but only in the presence of the G-domain of LAMA2, and for lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses. (genetex.com)
  • iii ) loss of LAMA2 from the basement membrane of the Schwann cells surrounding the peripheral nerves results in a lack of motor stimulation, leading to effective denervation atrophy. (pnas.org)
  • Defect of laminin M in skeletal and cardiac muscles and peripheral nerve of the homozygous dystrophic dy/dy mice. (springer.com)
  • mice
  • α2-Laminin extracted from the muscle of affected mice has considerably reduced polymerization activity ( Colognato and Yurchenco, 1999 ). (biologists.org)
  • Molecular
  • We used immunocytochemical and molecular studies of human myogenic cells and mouse muscles-comparing laminin-α2-deficient vs. (readbyqxmd.com)
  • vivo
  • The present application provides materials and methods for treating a patient with one or more conditions or disorders associated with LAMA2, both ex vivo or in vivo. (sumobrain.com)
  • adhesion
  • There, the laminins help regulate cell growth, cell movement (motility), and the attachment of cells to one another (adhesion). (nih.gov)
  • miRNAs
  • In order to identify LAMA2-CMD biomarkers we have profiled miRNAs in urine from the dy 2J / dy 2J mouse model of LAMA2-CMD at three distinct time points (representing asymptomatic, initial and established disease). (plos.org)
  • We suggest that urine miRNAs can be sensitive biomarkers for different stages of LAMA2-CMD. (plos.org)
  • myoblast
  • 1990 ). We have previously shown that myoblast survival and fusion into myotubes depends on laminin 111 (which is made up of α1, β1 and γ1 chains), and not on fibronectin or vitronectin (Clark et al. (springer.com)
  • Laminin 211 has also been reported to be important for myoblast fusion and myotube survival (Vachon et al. (springer.com)
  • Early muscle formation and myoblast fusion are normal, indicating that any deficiency in early Lama2 signaling does not lead to muscle pathology. (pnas.org)