Výsledky vyhledávání - "Bárbara Zambiasi Martinelli"

Characterization of joint disease in mucopolysaccharidosis type I mice

Autor: Luise Meurer, Ursula da Silveira Matte, Guilherme Baldo, Bárbara Zambiasi Martinelli, Roberto Giugliani, Fabiana Quoos Mayer, Ricardo Machado Xavier, Patricia Gnieslaw de Oliveira

Publikováno v: International Journal of Experimental Pathology. 94:305-311

Mucopolysaccharidoses (MPS) are lysosomal storage disorders characterized by mutations in enzymes that degrade glycosaminoglycans (GAGs). Joint disease is present in most forms of MPS, including MPS I. This work aimed to describe the joint disease pr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bd3f519e3eda4d577436754d83886519
https://doi.org/10.1111/iep.12033

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Subcutaneous implantation of microencapsulated cells overexpressing α-L-iduronidase for mucopolysaccharidosis type I treatment

Autor: Valeska Lizzi Lagranha, Giuseppe Ávila Testa, Talita Giacomet de Carvalho, Guilherme Baldo, Roberto Giugliani, Ursula da Silveira Matte, Bárbara Zambiasi Martinelli

Publikováno v: Journal of materials science. Materials in medicine. 28(3)

Mucopolysaccharidosis type I (MPS I) is caused by a deficiency of α-L-iduronidase (IDUA), resulting in accumulation of glycosaminoglycans (GAG) in lysosomes. Microencapsulation of recombinant cells is a promising gene/cell therapy approach that coul

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8ab80d578aa2ed50f9319859728ef4c2
https://pubmed.ncbi.nlm.nih.gov/28150116

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Intraperitoneal implant of recombinant encapsulated cells overexpressing alpha-l-iduronidase partially corrects visceral pathology in mucopolysaccharidosis type I mice

Autor: Bárbara Zambiasi Martinelli, Ursula da Silveira Matte, Fabiana Quoos Mayer, Fabíola Schons Meyer, Maira Graeff Burin, Angela Maria Vicente Tavares, Roberto Giugliani, Luise Meurer, Guilherme Baldo

Publikováno v: Cytotherapy. 14:860-867

Mucopolysaccharidosis type I (MPS I) is characterized by deficiency of the enzyme alpha-L-iduronidase (IDUA) and storage of glycosaminoglycans (GAG) in several tissues. Current available treatments present limitations, thus the search for new therapi

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0ba4e41fa30ed3551e0e80afd6c41080
https://doi.org/10.3109/14653249.2012.672730

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Deleterious effects of interruption followed by reintroduction of enzyme replacement therapy on a lysosomal storage disorder

Autor: Gabriela Pasqualim, Angela Maria Vicente Tavares, Ana Paula Krauthein Schneider, Bárbara Zambiasi Martinelli, Fabiana Quoos Mayer, Roberto Giugliani, Ursula da Silveira Matte, Guilherme Baldo, Graziela S. Ribas, Carmen Regla Vargas

Publikováno v: Translational research : the journal of laboratory and clinical medicine. 176

Temporary interruption of enzyme replacement therapy (ERT) in patients with different lysosomal storage disorders may happen for different reasons (adverse reactions, issues with reimbursement, logistic difficulties, and so forth), and the impact of

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::64b5c55ee99a227385405379b4575128
https://pubmed.ncbi.nlm.nih.gov/27450046

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Enzyme replacement therapy started at birth improves outcome in difficult-to-treat organs in mucopolysaccharidosis I mice

Autor: Guilherme Baldo, Talita Giacomet de Carvalho, Ângela Maria Vicente Tavares, Bárbara Zambiasi Martinelli, Fabíola Schons Meyer, Patricia Gnieslaw de Oliveira, Luise Meurer, Fabiana Quoos Mayer, Ursula da Silveira Matte, Roberto Giugliani

Publikováno v: Molecular genetics and metabolism. 109(1)

Since we previously observed that in patients with mucopolysaccharidosis (MPS) the storage of undegraded glycosaminoglycans (GAG) occurs from birth, in the present study we aimed to compare normal, untreated MPS I mice (knockout for alpha-l-iduronida

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::94bf4cc4c2dc4c845fc60f3d9d077154
https://pubmed.ncbi.nlm.nih.gov/23562162

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Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice

Autor: Fabiana Quoos Mayer, Fabíola Schons Meyer, Ursula da Silveira Matte, Katherine P. Ponder, Anna Cláudia Dilda, Roberto Giugliani, Guilherme Baldo, Bárbara Zambiasi Martinelli

Mucopolysaccharidosis (MPS) type I (Hurler syndrome) is a lysosomal storage disorder characterized by deficiency of alpha-L-iduronidase (IDUA), intracellular storage of glycosaminoglycans (GAGs) and progressive neurological pathology. The MPS I mouse

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b3763b8b371d0b8f4d4830f177c94135
https://europepmc.org/articles/PMC3390919/

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Enzyme Replacement Therapy in MPS I Mice: the Sooner The Better?

Autor: Luise Meurer, Roberto Giugliani, Fabiana Quoos Mayer, Angela Maria Vicente Tavares, Bárbara Zambiasi Martinelli, Talita Giacomet de Carvalho, Guilherme Baldo, Patricia Gnieslaw de Oliveira, Ursula da Silveira Matte, Fabíola Schons Meyer

Publikováno v: Molecular Genetics and Metabolism. 105:S31

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::bc02062c3d6a0eeb383c6239351a4434
https://doi.org/10.1016/j.ymgme.2011.11.062

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