Cell surface and gene expression regulation molecules in dystrophinopathy: mdx vs. Duchenne

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dc.contributor.authorFadic, R.
dc.date.accessioned2024-01-10T13:46:36Z
dc.date.available2024-01-10T13:46:36Z
dc.date.issued2005
dc.description.abstractDuchenne muscular dystrophy (DMD) is secondary to loss-of-function mutations in the dystrophin gene. The causes underlying the progression of DMD, differential muscle involvement, and the discrepancies in phenotypes among species with the same genetic defect are not understood. The mdx mouse, an animal model with dystrophin mutation has a milder phenotype. This article reviews the available information on expression of signaling-related molecules in DMD and mdx. Extracellular matrix proteoglycans, growth factors, integrins, caveolin-3, and neuronal nitric oxide synthase expression do not show significant differences. Calcineurin is inconsistently activated in mdx, which is associated with lack of cardiomyopathy, compared to the permanent calcineurin activation in mdx/utrophin null mice that have DMD-like cardiomyopathy. Levels of focal adhesion kinase (FAK) and extracellular regulated kinases (ERKs) differ among mdx and DMD. Further work is needed to identify the point of discrepancy in these signaling molecules' pathways in dystrophynopathies.
dc.fechaingreso.objetodigital2025-11-28
dc.format.extent6 páginas
dc.fuente.origenWOS
dc.identifier.issn0716-9760
dc.identifier.pubmedidMEDLINE:16579520
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/79179
dc.identifier.urihttp://dx.doi.org/10.4067/S0716-97602005000400010
dc.identifier.wosidWOS:000236190300010
dc.information.autorucMedicina;Fadic R;S/I;51912
dc.issue.numero4
dc.language.isoen
dc.nota.accesocontenido completo
dc.pagina.final380
dc.pagina.inicio375
dc.publisherSOCIEDAD BIOLGIA CHILE
dc.revistaBIOLOGICAL RESEARCH
dc.rightsacceso abierto
dc.subjectdystrophinopathy
dc.subjectsignaling molecules
dc.subjectDuchenne muscular dystrophy
dc.subjectFIBROBLAST-GROWTH-FACTOR
dc.subjectMUSCULAR-DYSTROPHY DMD
dc.subjectNITRIC-OXIDE SYNTHASE
dc.subjectSKELETAL-MUSCLE
dc.subjectTRANSFORMING GROWTH-FACTOR-BETA-1
dc.subjectALPHA-7-BETA-1 INTEGRIN
dc.subjectSIGNAL-TRANSDUCTION
dc.subjectSATELLITE CELLS
dc.subjectMOUSE MUSCLES
dc.subjectIN-VITRO
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleCell surface and gene expression regulation molecules in dystrophinopathy: mdx vs. Duchenne
dc.typeartículo
dc.volumen38
sipa.codpersvinculados51912
sipa.indexWOS
sipa.indexScielo
sipa.indexScopus
sipa.trazabilidadCarga SIPA;09-01-2024
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