No prominent role for complement C1-esterase inhibitor in Marfan syndrome mice

S Hibender, S Li, AV Postma, ME Hoogeland… - Vascular …, 2022 - vb.bioscientifica.com
Marfan syndrome (MFS) is a connective tissue disorder causing aortic aneurysm formation.
Currently, only prophylactic aortic surgery and blood pressure-lowering drugs are available …

Pharmacogenetic approach to losartan in Marfan patients: a starting point to improve dosing regimen?

FS Falvella, S Marelli, S Cheli, S Montanelli… - Drug metabolism and …, 2016 - degruyter.com
Background: Losartan is under evaluation for managing Marfan patients with aortic root
dilatation. Cytochrome P450 (CYP) enzymes convert losartan to E3174 active metabolite …

Preliminary analysis of the association of TRPV1 to the formation of Marfan syndrome aneurysms

ME Soto, E Soria Castro, V Guarner Lans… - 2019 - digitum.um.es
Marfan syndrome (MS) is an autosomal dominant disorder of connective tissue that is
caused by mutations in the fibrillin-1 (FBN-1) gene that cause degeneration of the artery. It is …

Marfan syndrome decreases Ca2+ wave frequency and vasoconstriction in murine mesenteric resistance arteries without changing underlying mechanisms

HT Syyong, AWY Chung, C Van Breemen - Journal of vascular research, 2011 - karger.com
Background/Aims: Vascular smooth muscle in Marfan syndrome, a connective tissue
disorder caused by mutations in FBN1 encoding fibrillin-1, is associated with decreased …

Cerebral Microvascular Density, Permeability of the Blood-Brain Barrier, and Neuroinflammatory Responses Indicate Early Aging Characteristics in a Marfan …

T Curry-Koski, L Curtin, M Esfandiarei, TC Thomas - bioRxiv, 2024 - biorxiv.org
Marfan Syndrome (MFS) is a connective tissue disorder due to mutations in fibrillin-1 (Fbn1),
where a Fbn1 missense mutation (Fbn1C1039G/+) can result in systemic increases in the …

Vascular dysfunctions in the isolated aorta of double-transgenic hypertensive mice developing aortic aneurysm

L Waeckel, C Badier-Commander, T Damery… - … -European Journal of …, 2015 - Springer
Angiotensin-II and oxidative stress are involved in the genesis of aortic aneurysms, a
phenomenon exacerbated by endothelial nitric oxide synthase (eNOS) deletion or …

Differentiation and quality control of smooth muscle cells from human pluripotent stem cells via the neural crest lineage

PJ Holt, H Davaapil, DK Shetty, AG Jacob, S Sinha - bioRxiv, 2023 - biorxiv.org
The Sinha laboratory has developed protocols for differentiating human pluripotent stem
cells (hPSCs) into vascular smooth muscle cells along developmental lineage-specific …

[PDF][PDF] PMC10924961.

T Curry, ME Barrameda, TC Thomas… - … BRAIN INJURY IN …, 2024 - repository.arizona.edu
In individuals with Marfan Syndrome (MFS), fibrillin-1 gene (Fbn1) mutations can lead to
vascular wall weakening and dysfunction. The experimental mouse model of MFS …

[PDF][PDF] Permeability of the Blood-Brain Barrier, Neuroinflammatory Responses, Altered Glutamate Neurotransmission, and Neurobehavioral Alterations Indicate Early …

TC Thomas, M Esfandiarei - FIBRILLIN-1 MUTATION …, 2024 - repository.arizona.edu
Marfan Syndrome (MFS) is a disorder of connective tissues due to mutations in the fibrillin-1
(Fbn1) gene. It has been shown that a missense mutation in the Fbn1 gene (Fbn1C1039G/+) …

[PDF][PDF] Permeability of the Blood-Brain Barrier, Neuroinflammatory Responses, Glutamate Neurotransmission, and Neurobehavioral Alterations After Acute Mild …

TC Thomas, M Esfandiarei - FIBRILLIN-1 MUTATION …, 2024 - repository.arizona.edu
Traumatic brain injury (TBI) can chronically increase transforming growth factor-β (TGF-β), a
cytokine implicated in cerebrovascular dysfunction underlying blood-brain barrier (BBB) …