Protein-Anchoring Therapy of Biglycan for Mdx Mouse Model of Duchenne Muscular Dystrophy

M Ito, Y Ehara, J Li, K Inada, K Ohno - Human gene therapy, 2017 - liebertpub.com
… of mdx mice upregulates expression of utrophin and other DAPC component proteins on the
sarcolemma, and ameliorates muscle pathology … a counter-equipped running wheel every 2 …

Non-glycanated biglycan and LTBP4: leveraging the extracellular matrix for Duchenne muscular dystrophy therapeutics

JR Fallon, EM McNally - Matrix Biology, 2018 - Elsevier
… Only NG-biglycan counters dystrophic pathology and … NG biglycan upregulated utrophin at
the sarcolemma motivated … of this protein to ameliorate dystrophic pathology in mdx mice. Two …

Sarcolemmal targeting of nNOSμ improves contractile function of mdx muscle

DL Rebolledo, MJ Kim, NP Whitehead… - Human molecular …, 2016 - academic.oup.com
sarcolemmal expression of utrophin and other members of the … for mdx-NOS-M muscle than
the other two groups of mdx mice, … of utrophin improves the dystrophic pathology in the mdx

Role of proteoglycans and glycosaminoglycans in Duchenne muscular dystrophy

L Carmen, V Maria, JC Morales-Medina… - …, 2019 - academic.oup.com
… Adult biglycan-null mice are mildly dystrophic displaying increased muscle fiber … rhBGN
upregulated utrophin at the sarcolemma and reduced muscle pathology in the mdx mouse model…

Celecoxib treatment improves muscle function in mdx mice and increases utrophin A expression

C Péladeau, NJ Adam, BJ Jasmin - The FASEB Journal, 2018 - Wiley Online Library
utrophin A expression in dystrophic muscles. Such methods include increasing the recruitment
of utrophin to the sarcolemma with biglycan … to their fast, glycolytic counterparts (66). Over …

[图书][B] Proteomic Characterisation of the mdx-4cv mouse model of Duchenne Muscular Dystrophy

S Murphy - 2018 - search.proquest.com
… and increases in the extracellular matrix stabilizers biglycan … samples from the dystrophic
mdx mouse and from biopsy … the sarcolemma through interactions with dystrophin-utrophin and …

Duchenne muscular dystrophy: pathogenesis and promising therapies

M Chang, Y Cai, Z Gao, X Chen, B Liu, C Zhang… - Journal of …, 2023 - Springer
Dystrophin connects the sarcolemma to the cytoskeleton of actin via the DAPC … utrophin
and DAPC-related component β-dystroglycan, and improves NMJ morphology in an mdx mouse […

Proteomic profiling of the dystrophin complex and membrane fraction from dystrophic mdx muscle reveals decreases in the cytolinker desmoglein and increases in …

S Murphy, H Brinkmeier, M Krautwald, M Henry… - Journal of Muscle …, 2017 - Springer
… to the dystrophin complex in the sarcolemma membrane. … -day-old mdx mouse model of
Duchenne muscular dystrophy (… of the inflammatory pathology of dystrophic muscles have …

[HTML][HTML] Promising therapeutic approaches of utrophin replacing dystrophin in the treatment of Duchenne muscular dystrophy

R Wu, Y Song, S Wu, Y Chen - Fundamental Research, 2022 - Elsevier
dystrophin recovery effects in the sarcolemma of mdx mouse … -102 is a recombinant biglycan
that can improve muscle health, … utrophin as well as the muscle strength and pathology of …

[HTML][HTML] Current translational research and murine models for Duchenne muscular dystrophy

M Rodrigues, Y Echigoya, S Fukada… - Journal of …, 2016 - content.iospress.com
Biglycan, is a proteoglycan found endogenously in mice and … membrane by recruiting
utrophin to the sarcolemma [90]. … utrophin and reduces muscular dystrophy in mdx mice [91]. …