grant
Muscular Dystrophy Specialized Research Center
Organization UNIVERSITY OF IOWALocation IOWA CITY, UNITED STATESPosted 8 Jun 2005Deadline 30 Jun 2030
NIHUS FederalResearch GrantFY2025AffectApneaAssayBindingBioassayBiochemicalBiological AssayBody TissuesClinical TrialsCraninDNA mutationDefectDevelopmentDysfunctionDystroglycanECM receptorEffectivenessEnzyme GeneEnzymesExtracellular Matrix ProteinsFKRPFoundationsFunctional disorderGenesGenetic ChangeGenetic defectGenetic mutationGenetic studyGlycoprotein GP-2HypercapniaHypoxiaHypoxicImpairmentIndividualKnowledgeLGMD2ILamininLengthLimb-Girdle Muscular DystrophiesMeasuresMiceMice MammalsMolecularMolecular InteractionMonitorMurineMusMuscular DystrophiesMutationMyodystrophicaMyodystrophyNational Institutes of HealthOutcomeOxygen DeficiencyPathologicPatientsPhysiologicPhysiologicalPhysiopathologyPlethysmographyProcessPropertyProteinsRegulationResearchResearch PriorityRespiration DisordersRespiratory DisorderRespiratory MusclesRespiratory physiologySiteSupplementationTestingTherapeuticTidal VolumeTissuesUnited States National Institutes of HealthValidationVentilatory Musclesbreathing disordercarbon dioxide retentiondesigndesigningdetermine efficacydevelopmentaldystroglycanopathyefficacy analysisefficacy assessmentefficacy determinationefficacy evaluationefficacy examinationelevated carbon dioxideenzyme substrate complexevaluate efficacyexamine efficacyexperienceextracellular matrix receptorfukutin related proteingene replacementgenome mutationhypercarbiaimprovedin vivoincreased level Carbon dioxidelimb-girdle muscular weakness and atrophylimb-girdle syndromemouse modelmurine modelmuscle dystrophymyopathic limb-girdle syndromenovelpathophysiologyreceptor functionrespiratoryrespiratory airway volumerespiratory dysfunctionrespiratory functionresponseskeletal muscle weaknesssugarvalidationsventilationα-DGα-DystroglycanαDG
Description preview
Project 1 Project Summary
Dystroglycanopathies are a group of congenital/limb-girdle muscular dystrophies that lead to progressive
skeletal muscle weakness and a decline in respiratory function. Defects in the post-translational processing of
the extracellular matrix (ECM) receptor, α-dystroglycan (α-DG), cause dystroglycanopathies. ECM proteins…
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