Abnormal distribution of inositol 1,4,5-trisphosphate receptors in human muscle can be related to altered calcium signals and gene expression in Duchenne dystrophy-derived cells

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dc.contributor.authorCardenas, Cesar
dc.contributor.authorJuretic, Nevenka
dc.contributor.authorBevilacqua, Jorge A.
dc.contributor.authorGarcia, Isaac E.
dc.contributor.authorFigueroa, Reinaldo
dc.contributor.authorHartley, Ricardo
dc.contributor.authorTaratuto, Ana L.
dc.contributor.authorGejman, Roger
dc.contributor.authorRiveros, Nora
dc.contributor.authorMolgo, Jordi
dc.contributor.authorJaimovich, Enrique
dc.date.accessioned2024-01-10T13:45:31Z
dc.date.available2024-01-10T13:45:31Z
dc.date.issued2010
dc.description.abstractInositol 1,4,5-trisphosphate (IP3) receptors (IP(3)Rs) drive calcium signals involved in skeletal muscle excitation-transcription coupling and plasticity; IP3R subtype distribution and downstream events evoked by their activation have not been studied in human muscle nor has their possible alteration in Duchenne muscular dystrophy (DMD). We studied the expression and localization of IP3R subtypes in normal and DMD human muscle and in normal (RCMH) and dystrophic (RCDMD) human muscle cell lines. In normal muscle, both type 1 IP(3)Rs (IP(3)R1) and type 2 IP(3)Rs (IP(3)R2) show a higher expression in type II fibers, whereas type 3 IP(3)Rs (IP(3)R3) show uniform distribution. In DMD biopsies, all fibers display a homogeneous IP(3)R2 label, whereas 24 +/- 7% of type II fibers have lost the IP(3)R1 label. RCDMD cells show 5-fold overexpression of IP(3)R2 and down-regulation of IP(3)R3 compared with RCMH cells. A tetanic stimulus induces IP3-dependent slow Ca2+ transients significantly larger and faster in RCDMD cells than in RCMH cells as well as significant ERK1/2 phosphorylation in normal but not in dystrophic cells. Excitation-driven gene expression was different among cell lines; 44 common genes were repressed in RCMH cells and expressed in RCDMD cells or vice versa. IP3-dependent Ca2+ release may play a significant role in DMD pathophysiology.-Cardenas, C., Juretic, N., Bevilacqua, J. A., Garcia, I. E., Figueroa, R., Hartley, R., Taratuto, A. L., Gejman, R., Riveros, N., Molgo, J., Jaimovich, E. Abnormal distribution of inositol 1,4,5-trisphosphate receptors in human muscle can be related to altered calcium signals and gene expression in Duchenne dystrophy-derived cells. FASEB J. 24, 3210-3221 (2010). www.fasebj.org
dc.description.funderFondo de Investigacion Avanzada en Areas Prioritarias (FONDAP)
dc.description.funderFondo Nacional de Investigacion Cientifica y Tecnologica
dc.description.funderECOS Sud-CONICYT exchange program
dc.description.funderProyecto OAIC
dc.description.funderHospital Clinico Universidad de Chile
dc.description.funderAssociation Francaise Contre les Myopathies
dc.description.funderFONDAP
dc.description.funderAFM at Laboratoire de Neurobiologie Cellulaire et Moleculaire (France)
dc.description.funderPrograma Bicentenario de Ciencia y Tecnologia
dc.fechaingreso.objetodigital2024-05-02
dc.format.extent12 páginas
dc.fuente.origenWOS
dc.identifier.doi10.1096/fj.09-152017
dc.identifier.eissn1530-6860
dc.identifier.issn0892-6638
dc.identifier.pubmedidMEDLINE:20395455
dc.identifier.urihttps://doi.org/10.1096/fj.09-152017
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/79044
dc.identifier.wosidWOS:000281446400010
dc.information.autorucMedicina;Gejman R;S/I;900
dc.issue.numero9
dc.language.isoen
dc.nota.accesocontenido parcial
dc.pagina.final3221
dc.pagina.inicio3210
dc.publisherFEDERATION AMER SOC EXP BIOL
dc.revistaFASEB JOURNAL
dc.rightsacceso restringido
dc.subjectskeletal muscle
dc.subjectexcitation-transcription coupling
dc.subjectdystrophin
dc.subjectmuscle plasticity
dc.subjectmyofiber types
dc.subjectELEMENT-BINDING PROTEIN
dc.subjectSKELETAL-MUSCLE
dc.subjectMUSCULAR-DYSTROPHY
dc.subjectCA2+ TRANSIENTS
dc.subjectUP-REGULATION
dc.subjectMEMBRANE DEPOLARIZATION
dc.subjectCARDIAC MYOCYTES
dc.subjectSATELLITE CELLS
dc.subjectIP3 RECEPTORS
dc.subjectMDX MUSCLE
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleAbnormal distribution of inositol 1,4,5-trisphosphate receptors in human muscle can be related to altered calcium signals and gene expression in Duchenne dystrophy-derived cells
dc.typeartículo
dc.volumen24
sipa.codpersvinculados900
sipa.indexWOS
sipa.indexScopus
sipa.trazabilidadCarga SIPA;09-01-2024
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