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Alzecure Foundation Huddinge Sweden 1 Alzecure Pharma Huddinge Sweden 1 Clinical Neurochemistry Laboratory Sa... 1 Department of Cell Biology Faculty of... 1 Department of Cell and Molecular Biol... 1 Department of Clinical Genetics Leide... 1 Department of Neurobiology Care Scien... 1 Department of Neurodegenerative Disea... 1 Department of Psychiatry and Neuroche... 1 Hong Kong Center for Neurodegenerativ... 1 Sinfonia Biotherapeutics Huddinge Sweden 1 UK Dementia Research Institute at UCL... 1
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Alzecure Foundation Huddinge Sweden 1 Alzecure Pharma Huddinge Sweden 1 Clinical Neurochemistry Laboratory Sa... 1 Department of Cell Biology Faculty of... 1 Department of Cell and Molecular Biol... 1 Department of Clinical Genetics Leide... 1 Department of Neurobiology Care Scien... 1 Department of Neurodegenerative Disea... 1 Department of Psychiatry and Neuroche... 1 Hong Kong Center for Neurodegenerativ... 1 Sinfonia Biotherapeutics Huddinge Sweden 1 UK Dementia Research Institute at UCL... 1
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- Oliveira, Daniel V
- Coupland, Kirsten G
- Shao, Wenchao
- Jin, Shaobo
- Del Gaudio, Francesca
- Wang, Sailan
- Fox, Rhys
- Rutten, Julie W
- Sandin, Johan
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Zetterberg, Henrik
Autor Zetterberg, Henrik Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, The Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital, Mölndal, Sweden Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, UK UK Dementia Research Institute at UCL, London, UK Hong Kong Center for Neurodegenerative Diseases, Clear Water Bay, Hong Kong, China
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PubMed
36524456
DOI
10.15252/emmm.202216556
Knihovny.cz E-zdroje
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic form of familial small vessel disease; no preventive or curative therapy is available. CADASIL is caused by mutations in the NOTCH3 gene, resulting in a mutated NOTCH3 receptor, with aggregation of the NOTCH3 extracellular domain (ECD) around vascular smooth muscle cells. In this study, we have developed a novel active immunization therapy specifically targeting CADASIL-like aggregated NOTCH3 ECD. Immunizing CADASIL TgN3R182C150 mice with aggregates composed of CADASIL-R133C mutated and wild-type EGF1-5 repeats for a total of 4 months resulted in a marked reduction (38-48%) in NOTCH3 deposition around brain capillaries, increased microglia activation and lowered serum levels of NOTCH3 ECD. Active immunization did not impact body weight, general behavior, the number and integrity of vascular smooth muscle cells in the retina, neuronal survival, or inflammation or the renal system, suggesting that the therapy is tolerable. This is the first therapeutic study reporting a successful reduction of NOTCH3 accumulation in a CADASIL mouse model supporting further development towards clinical application for the benefit of CADASIL patients.
- MeSH
- aktivní imunoterapie MeSH
- CADASIL * genetika terapie MeSH
- kapiláry metabolismus MeSH
- modely nemocí na zvířatech MeSH
- mozek metabolismus MeSH
- mutace MeSH
- myši MeSH
- receptor Notch3 genetika metabolismus MeSH
- receptory Notch metabolismus MeSH
- zvířata MeSH
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- myši MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
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