Immune-mediated mechanisms potentially regulate the disease time-course of duchenne muscular dystrophy and provide targets for therapeutic intervention

NP Evans, SA Misyak, JL Robertson… - PM&R, 2009 - Elsevier
Duchenne muscular dystrophy is a lethal muscle-wasting disease that affects boys.
Mutations in the dystrophin gene result in the absence of the dystrophin glycoprotein …

[HTML][HTML] T-cell-dependent fibrosis in the mdx dystrophic mouse

J Morrison, QL Lu, C Pastoret, T Partridge… - Laboratory …, 2000 - Elsevier
In Duchenne muscular dystrophy patients, the pathological hallmark of the disease, namely,
the chronic accumulation of sclerotic scar tissue in the interstitial space of skeletal muscle is …

Resolution of skeletal muscle inflammation in mdx dystrophic mouse is accompanied by increased immunoglobulin and interferon‐γ production

J Lagrota‐Candido, R Vasconcellos… - International journal …, 2002 - Wiley Online Library
Mdx mouse, the animal model of Duchenne muscular dystrophy, develops an X‐linked
recessive inflammatory myopathy with an apparent sustained capacity for muscle …

Regenerated mdx mouse skeletal muscle shows differential mRNA expression

BS Tseng, P Zhao, JS Pattison… - Journal of applied …, 2002 - journals.physiology.org
Despite over 3,000 articles published on dystrophin in the last 15 years, the reasons
underlying the progression of the human disease, differential muscle involvement, and …

Gender dimorphism influences extracellular matrix expression and regeneration of muscular tissue in mdx dystrophic mice

MC Salimena, J Lagrota-Candido… - Histochemistry and cell …, 2000 - Springer
Mdx mouse, the animal model of Duchenne muscular dystrophy, lacks dystrophin and
develops an X-linked recessive inflammatory myopathy characterized by degeneration of …

A phase 2 open-label study of the safety and efficacy of weekly dosing of ATL1102 in patients with non-ambulatory Duchenne muscular dystrophy and pharmacology …

IR Woodcock, G Tachas, N Desem, PJ Houweling… - Plos one, 2024 - journals.plos.org
Background ATL1102 is a 2'MOE gapmer antisense oligonucleotide to the CD49d alpha
subunit of VLA-4, inhibiting expression of CD49d on lymphocytes, reducing survival …

Pattern of metalloprotease activity and myofiber regeneration in skeletal muscles of mdx mice

C Bani, J Lagrota‐Candido, DF Pinheiro… - Muscle & Nerve …, 2008 - Wiley Online Library
Matrix metalloproteases (MMPs) are key regulatory molecules in the formation, remodeling,
and degradation of extracellular matrix components in both physiological and pathological …

CD49d is a disease progression biomarker and a potential target for immunotherapy in Duchenne muscular dystrophy

F Pinto-Mariz, L Rodrigues Carvalho… - Skeletal muscle, 2015 - Springer
Background Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin
gene. The immune inflammatory response also contributes to disease progression in DMD …

A Phase 2 open-label study to determine the safety and efficacy of weekly dosing of ATL1102 in patients with non-ambulatory Duchenne muscular dystrophy

IR Woodcock, G Tachas, N Desem, PJ Houweling… - medRxiv, 2022 - medrxiv.org
Background ATL1102 is a 2'MOE gapmer antisense oligonucleotide to the CD49d alpha
subunit of VLA-4. ATL1102 inhibits expression of CD49d on lymphocytes, thereby reducing …

Recent advances and new insights into muscular lymphangiogenesis in health and disease

RC Ji - Life sciences, 2018 - Elsevier
The lymphatic vessels have been implicated in maintenance of interstitial fluid homeostasis
and immune responses, and pathological conditions including inflammation, wound healing …