Proteínas reguladoras do cálcio e proteção à mionecrose na distrofia muscular de Duchenne

• Processo: 04/15526-9
• Modalidade de apoio: Auxílio à Pesquisa - Regular
• Vigência: 01 de junho de 2005 - 31 de agosto de 2008
• Área do conhecimento: Ciências Biológicas - Morfologia - Anatomia
• Pesquisador responsável: Maria Julia Marques
• Beneficiário: Maria Julia Marques
• Instituição Sede: Instituto de Biologia (IB). Universidade Estadual de Campinas (UNICAMP). Campinas , SP, Brasil
• Assunto(s): Junção neuromuscular Distrofia muscular de Duchenne Cálcio Microscopia confocal Ações farmacológicas
• Palavra(s)-Chave do Pesquisador: Calcio | Distrofia Muscular | Juncao Neuromuscular | Mdx | Microscopia Confocal | Musculo Extra-Ocular

Resumo

No camundongo mdx, modelo experimental da distrofia muscular, os músculos extra-oculares não sofrem mionecrose, tornando-os uma ferramenta importante para melhor compreensão dos mecanismos da patogênese da distrofia e a busca de novos medicamentos para a terapia farmacológica desta patologia. No presente trabalho iremos verificar se alterações nos níveis de proteínas reguladoras do cálcio podem se relacionar ao mecanismo de proteção contra a mionecrose observado nos músculos extra-oculares de camundongos mdx. Através de técnicas de imunoblotting e imunohistoquímica iremos verificar se a expressão e distribuição da calmodulina, calsequestrina, calpaina e Serca estão alteradas nestes músculos, quando comparada com músculos da pata. Adicionalmente, através de microscopia confocal de fluorescência, iremos verificar se alterações no padrão de distribuição dos receptores de acetilcolina e dos terminais nervosos, observadas em junções neuromusculares distróficas, são decorrentes da falta da distrofina ou da regeneração da fibra muscular. (AU)

Publicações científicas

(20)

(Referências obtidas automaticamente do Web of Science e do SciELO, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores)

MOREIRA, DRIELEN DE OLIVEIRA; PEREIRA, JULIANO ALVES; TIEMI TANIGUTI, ANA PAULA; MATSUMURA, CINTIA YURI; FERREIRA RAMOS, LUIS ALBERTO; AREAS, MIGUEL ARCANJO; NETO, HUMBERTO SANTO; MARQUES, MARIA JULIA. SURAMIN ATTENUATES DYSTROPHIN-DEFICIENT CARDIOMYOPATHY IN THE mdx MOUSE MODEL OF DUCHENNE MUSCULAR DYSTROPHY. MUSCLE & NERVE, v. 48, n. 6, p. 911-919, DEC 2013. (12/03498-7, 04/15526-9, 08/58491-1, 11/51697-6, 08/54775-5)

MARQUES‚ M.J.; FERRETTI‚ R.; VOMERO‚ V.U.; MINATEL‚ E.; NETO‚ H.S.. Intrinsic laryngeal muscles are spared from myonecrosis in the mdx mouse model of Duchenne muscular dystrophy. MUSCLE & NERVE, v. 35, n. 3, p. 349-353, 2007. (01/00570-4, 04/15526-9)

FERRETTI‚ R.; MARQUES‚ M.J.; PERTILLE‚ A.; SANTO NETO‚ H.. Sarcoplasmic-endoplasmic-reticulum Ca2+-ATPase and calsequestrin are overexpressed in spared intrinsic laryngeal muscles of dystrophin-deficient mdx mice. MUSCLE & NERVE, v. 39, n. 5, p. 609-615, 2009. (01/00570-4, 04/15526-9)

MARQUES, MARIA JULIA; OGGIAM, DANIELLA SILVA; CHAGAS BARBIN, ISABEL CRISTINA; FERRETTI, RENATO; SANTO NETO, HUMBERTO. LONG-TERM THERAPY WITH DEFLAZACORT DECREASES MYOCARDIAL FIBROSIS IN mdx MICE. MUSCLE & NERVE, v. 40, n. 3, p. 466-468, SEP 2009. (01/00570-4, 04/15526-9)

PEREIRA, JULIANO ALVES; TIEMI TANIGUTI, ANA PAULA; MATSUMURA, CINTIA; MARQUES, MARIA JULIA; NETO, HUMBERTO SANTO. Doxycycline ameliorates the dystrophic phenotype of skeletal and cardiac muscles in mdx mice. MUSCLE & NERVE, v. 46, n. 3, p. 400-406, SEP 2012. (08/58491-1, 08/54775-5, 04/15526-9)

PEREIRA, JULIANO ALVES; MAURICIO, ADRIANA FOGAGNOLO; MARQUES, MARIA JULIA; NETO, HUMBERTO SANTO. Dual Therapy Deflazacort/Doxycyclyne Is Better Than Deflazacort Monotherapy to Alleviate Cardiomyopathy in Dystrophin-Deficient mdx Mice. JOURNAL OF CARDIOVASCULAR PHARMACOLOGY AND THERAPEUTICS, v. 22, n. 5, p. 458-466, SEP 2017. (11/51697-6, 08/58491-1, 12/13577-1, 04/15526-9)

MARANHAO, JULIANA BARROS; MOREIRA, DRIELEN DE OLIVEIRA; MAURICIO, ADRIANA FOGAGNOLO; DE CARVALHO, SAMARA CAMACARI; FERRETTI, RENATO; PEREIRA, JULIANO ALVES; SANTO NETO, HUMBERTO; MARQUES, MARIA JULIA. Changes in calsequestrin, TNF-alpha, TGF-beta and MyoD levels during the progression of skeletal muscle dystrophy in mdx mice: a comparative analysis of the quadriceps, diaphragm and intrinsic laryngeal muscles. International Journal of Experimental Pathology, v. 96, n. 5, p. 285-293, OCT 2015. (12/03498-7, 04/15526-9, 08/58491-1, 11/51697-6, 12/13577-1, 12/15492-3, 14/04782-6)

PEREIRA, JULIANO ALVES; MARQUES, MARIA JULIA; SANTO NETO, HUMBERTO. Co-administration of deflazacort and doxycycline: a potential pharmacotherapy for Duchenne muscular dystrophy. Clinical and Experimental Pharmacology and Physiology, v. 42, n. 7, p. 788-794, JUL 2015. (11/51697-6, 08/58491-1, 14/04782-6, 04/15526-9)

MARQUES‚ M.J.; MACHADO‚ R.V.; MINATEL‚ E.; NETO‚ H.S.. Disodium cromoglycate protects dystrophin-deficient muscle fibers from leakiness. MUSCLE & NERVE, v. 37, n. 1, p. 61-67, 2008. (01/00570-4, 04/15526-9)

MARQUES‚ M.J.; PEREIRA‚ E.C.L.; MINATEL‚ E.; NETO‚ H.S.. Nerve-terminal and Schwann-cell response after nerve injury in the absence of nitric oxide. MUSCLE & NERVE, v. 34, n. 2, p. 225-231, 2006. (01/00570-4, 04/15526-9)

MARQUES‚ M.J.; MATSUMURA‚ C.Y.; SANTO NETO‚ H.. Alterations in the permeability of dystrophic fibers during neuromuscular junction development. ACTA BIOLOGICA HUNGARICA, v. 58, n. 1, p. 1-9, 2007. (01/00570-4, 04/15526-9, 02/08145-3)

MATSUMURA‚ C.Y.; PERTILLE‚ A.; ALBUQUERQUE‚ T.C.P.; SANTO NETO‚ H.; MARQUES‚ M.J.. Diltiazem and verapamil protect dystrophin-deficient muscle fibers of MDX mice from degeneration: A potential role in calcium buffering and sarcolemmal stability. MUSCLE & NERVE, v. 39, n. 2, p. 167-176, 2009. (01/00570-4, 04/15526-9, 06/54114-3)

CHAGAS BARBIN, ISABEL CRISTINA; PEREIRA, JULIANO ALVES; ROVERE, MATHEUS BERSAN; MOREIRA, DRIELEN DE OLIVEIRA; MARQUES, MARIA JULIA; SANTO NETO, HUMBERTO. Diaphragm degeneration and cardiac structure in mdx mouse: potential clinical implications for Duchenne muscular dystrophy. Journal of Anatomy, v. 228, n. 5, p. 784-791, MAY 2016. (08/58491-1, 14/04782-6, 04/15526-9)

FERRETTI, RENATO; MARQUES, MARIA JULIA; KHURANA, TEJVIR S.; NETO, HUMBERTO SANTO. Expression of calcium-buffering proteins in rat intrinsic laryngeal muscles. PHYSIOLOGICAL REPORTS, v. 3, n. 6, JUN 2015. (08/58491-1, 13/10633-0, 04/15526-9)

PEREIRA, JULIANO ALVES; MATSUMURA, CINTIA YURI; MINATEL, ELAINE; MARQUES, MARIA JULIA; NETO, HUMBERTO SANTO. UNDERSTANDING THE BENEFICIAL EFFECTS OF DOXYCYCLINE ON THE DYSTROPHIC PHENOTYPE OF THE mdx MOUSE. MUSCLE & NERVE, v. 50, n. 2, p. 283-286, AUG 2014. (11/51697-6, 08/54775-5, 08/58491-1, 04/15526-9)

MATSUMURA, CINTIA YURI; TIEMI TANIGUTI, ANA PAULA; PERTILLE, ADRIANA; SANTO NETO, HUMBERTO; MARQUES, MARIA JULIA. Stretch-activated calcium channel protein TRPC1 is correlated with the different degrees of the dystrophic phenotype in mdx mice. AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY, v. 301, n. 6, p. C1344-C1350, DEC 2011. (08/58491-1, 08/54775-5, 04/15526-9)

FERRETTI, RENATO; SANTO NETO, HUMBERTO; MARQUES, MARIA JULIA. Expression of Utrophin at Dystrophin-Deficient Neuromuscular Synapses of mdx Mice: A Study of Protected and Affected Muscles. Anatomical Record-Advances in Integrative Anatomy and Evolutionary Biology, v. 294, n. 2, p. 283-286, FEB 2011. (08/58491-1, 04/15526-9)

MARQUES, MARIA JULIA; CHAGAS BARBIN, ISABEL CRISTINA; TIEMI TANIGUTI, ANA PAULA; OGGIAN, DANIELA SILVA; FERRETTI, R.; SANTO NETO, H.. MYOCARDIAL FIBROSIS IS UNALTERED BY LONG-TERM ADMINISTRATION OF L-ARGININE IN DYSTROPHIN DEFICIENT MDX MICE: A HISTOMORPHOMETRIC ANALYSIS. ACTA BIOLOGICA HUNGARICA, v. 61, n. 2, p. 168-174, JUN 2010. (01/00570-4, 04/15526-9)

GUIDO, ANDERSON NERI; ROCHA CAMPOS, GERSON EDUARDO; SANTO NETO, HUMBERTO; MARQUES, MARIA JULIA; MINATEL, ELAINE. Fiber Type Composition of the Sternomastoid and Diaphragm Muscles of Dystrophin-Deficient mdx Mice. Anatomical Record-Advances in Integrative Anatomy and Evolutionary Biology, v. 293, n. 10, p. 1722-1728, OCT 2010. (07/50189-1, 08/50731-3, 08/58491-1, 04/15526-9)

PERTILLE, ADRIANA; TONIZZA DE CARVALHO, CANDIDA LUIZA; MATSUMURA, CINTIA YURI; SANTO NETO, HUMBERTO; MARQUES, MARIA JULIA. Calcium-binding proteins in skeletal muscles of the mdx mice: potential role in the pathogenesis of Duchenne muscular dystrophy. International Journal of Experimental Pathology, v. 91, n. 1, p. 63-71, FEB 2010. (01/00570-4, 04/15526-9)