Výsledky vyhledávání - "Zandra Körner"

Akademický článek

Bortezomib Does Not Reduce Muscular Dystrophy in the dy2J/dy2J Mouse Model of Laminin α2 Chain-Deficient Muscular Dystrophy.

Autor: Zandra Körner, Madeleine Durbeej

Publikováno v: PLoS ONE, Vol 11, Iss 1, p e0146471 (2016)

Congenital muscular dystrophy with laminin α2 chain-deficiency, also known as MDC1A, is a severe neuromuscular disorder for which there is no cure. Patients with complete laminin α2 chain-deficiency typically have an early onset disease with a more

Externí odkaz: https://doaj.org/article/f9f5a836573c444faf9f56f249149acf

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Early skeletal muscle pathology and disease progress in the dy 3K /dy 3K mouse model of congenital muscular dystrophy with laminin α2 chain-deficiency

Autor: Bruno Menezes de Oliveira, Zandra Körner, Madeleine Durbeej, Kinga I. Gawlik

Publikováno v: Scientific Reports, Vol 9, Iss 1, Pp 1-17 (2019)
Scientific Reports

Deficiency of laminin α2 chain leads to a severe form of congenital muscular dystrophy (LAMA2-CMD), and dystrophic symptoms progress rapidly in early childhood. Currently, there is no treatment for this detrimental disorder. Development of therapies

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c905f3be654dc3176d34680fd5c042e
http://link.springer.com/article/10.1038/s41598-019-50550-0

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Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study

Autor: Zandra Körner, Madeleine Durbeej, Bernardo Moreira Soares Oliveira, Cibely C. Fontes-Oliveira, Vahid M. Harandi

Publikováno v: Scientific Reports
Scientific Reports, Vol 8, Iss 1, Pp 1-15 (2018)

Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe muscle disorder with complex underlying pathogenesis. We have previously employed profiling techniques to elucidate molecular patterns and demonstrated significan

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::07ca2b21ab82da0cca7786c50eef8492
http://europepmc.org/articles/PMC6214987

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Bortezomib Partially Improves Laminin α2 Chain–Deficient Muscular Dystrophy

Autor: Virginie Carmignac, Zandra Körner, Madeleine Durbeej, Johan Holmberg, Cibely C. Fontes-Oliveira

Publikováno v: The American Journal of Pathology. 184:1518-1528

Congenital muscular dystrophy, caused by mutations in LAMA2 (the gene encoding laminin α2 chain), is a severe and incapacitating disease for which no therapy is yet available. We have recently demonstrated that proteasome activity is increased in la

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::be477aec2cef4c174ace7e1e34533aa0
https://doi.org/10.1016/j.ajpath.2014.01.019

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Bortezomib Does Not Reduce Muscular Dystrophy in the dy2J/dy2J Mouse Model of Laminin α2 Chain-Deficient Muscular Dystrophy

Autor: Madeleine Durbeej, Zandra Körner

Publikováno v: PLoS ONE
PLoS ONE, Vol 11, Iss 1, p e0146471 (2016)

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bf534ebccb8851fc68fb296a6afa4cac
https://pubmed.ncbi.nlm.nih.gov/26731667

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Autophagy is increased in laminin α2 chain-deficient muscle and its inhibition improves muscle morphology in a mouse model of MDC1A

Autor: Madeleine Durbeej, Cintia Yuri Matsumura, Kinga I. Gawlik, Martina Svensson, Linda Elowsson, Zandra Körner, Virginie Carmignac, Valérie Allamand

Publikováno v: Human molecular genetics. 20(24)

Congenital muscular dystrophy caused by laminin α2 chain deficiency (also known as MDC1A) is a severe and incapacitating disease, characterized by massive muscle wasting. The ubiquitin-proteasome system plays a major role in muscle wasting and we re

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c752c12602210ed73920e97806bf112
https://pubmed.ncbi.nlm.nih.gov/21920942

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