Novel Murine Models of Cerebral Cavernous Malformations.
1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine
/ analogs & derivatives
Acute Disease
Animals
Apoptosis Regulatory Proteins
/ deficiency
Brain
/ blood supply
Cholecalciferol
/ pharmacology
Chronic Disease
Cyclic N-Oxides
/ pharmacology
Disease Models, Animal
Gene Deletion
Hemangioma, Cavernous, Central Nervous System
/ complications
Hemorrhage
/ complications
Lipopolysaccharides
Mice, Inbred C57BL
Models, Biological
Phenotype
Spin Labels
CCM
Cavernous angioma
Cerebral cavernous malformation
Cerebral hemorrhage
Fasudil
Lipopolysaccharide
Stroke
Tempol
Vitamin D
Journal
Angiogenesis
ISSN: 1573-7209
Titre abrégé: Angiogenesis
Pays: Germany
ID NLM: 9814575
Informations de publication
Date de publication:
11 2020
11 2020
Historique:
received:
10
03
2020
accepted:
06
07
2020
pubmed:
28
7
2020
medline:
29
9
2021
entrez:
26
7
2020
Statut:
ppublish
Résumé
Cerebral cavernous malformations (CCMs) are ectatic capillary-venous malformations that develop in approximately 0.5% of the population. Patients with CCMs may develop headaches, focal neurologic deficits, seizures, and hemorrhages. While symptomatic CCMs, depending upon the anatomic location, can be surgically removed, there is currently no pharmaceutical therapy to treat CCMs. Several mouse models have been developed to better understand CCM pathogenesis and test therapeutics. The most common mouse models induce a large CCM burden that is anatomically restricted to the cerebellum and contributes to lethality in the early days of life. These inducible models thus have a relatively short period for drug administration. We developed an inducible CCM3 mouse model that develops CCMs after weaning and provides a longer period for potential therapeutic intervention. Using this new model, three recently proposed CCM therapies, fasudil, tempol, vitamin D
Identifiants
pubmed: 32710309
doi: 10.1007/s10456-020-09736-8
pii: 10.1007/s10456-020-09736-8
pmc: PMC7530029
mid: NIHMS1614973
doi:
Substances chimiques
Apoptosis Regulatory Proteins
0
Cyclic N-Oxides
0
Lipopolysaccharides
0
PDCD10 protein, mouse
0
Spin Labels
0
Cholecalciferol
1C6V77QF41
1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine
84477-87-2
fasudil
Q0CH43PGXS
tempol
U78ZX2F65X
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
651-666Subventions
Organisme : American Heart Association-American Stroke Association
ID : 18PRE34060061
Pays : United States
Organisme : NHLBI NIH HHS
ID : F30 HL140871
Pays : United States
Organisme : NINDS NIH HHS
ID : P01 NS092521
Pays : United States
Organisme : NIGMS NIH HHS
ID : T32 GM007171
Pays : United States
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