Results 11 to 20 of about 8,915 (192)

Is the oxidant/antioxidant status altered in CADASIL patients? [PDF]

PLoS ONE, 2013
The altered aggregation of proteins in non-native conformation is associated with endoplasmic reticulum derangements, mitochondrial dysfunction and excessive production of reactive oxygen species. Cerebral autosomal dominant arteriopathy with subcortical
Jonica Campolo, Renata De Maria, Caterina Mariotti, Chiara Tomasello, Marina Parolini, Marina Frontali, Domenico Inzitari, Raffaella Valenti, Antonio Federico, Franco Taroni, Oberdan Parodi   +10 more
doaj   +14 more sources

Characteristics and Long‐Term Outcome of Acute Ischemic Stroke in Patients With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Compared With Sporadic Small Vessel Occlusion [PDF]

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) often presents as acute ischemic stroke involving cerebral perforators.
Sang Hee Ha, Jae Young Park, Sung Hee Ahn, Jun Young Chang, Chong Hyun Suh, Hyunjin Kim, Jae‐Sung Lim, Dong‐Wha Kang, Sun U. Kwon, Satoshi Saito, Bum Joon Kim   +10 more
doaj   +2 more sources

NOTCH3 CADASIL Variant Receptor Aggregation Requires NOTCH3 Wild-Type Receptors: Identification of Highly Selective Inhibitors That Block the Process. [PDF]

FASEB J
CADASIL is the major cause of early‐onset stroke and cognitive dysfunction, including dementia. It is caused by mutations in the NOTCH3 receptor that result in the formation of protein aggregates in the small vessel walls of the brain. We demonstrated that NOTCH3 CADASIL variant receptor aggregation is strictly dependent on interactions with NOTCH3 ...
Wang H, Liu X, Gravesteijn G, Atella A, Liu J, Rutten JW, Ten Dijke P, Gonçalves MAFV, Lesnik Oberstein SAJ, Baker DA.   +9 more
europepmc   +2 more sources

Study of the NOTCH3 Gene Reveals the First CADASIL Cases in Crete and a Novel Pathogenic Variant [PDF]

Brain and Behavior
Background NOTCH3 gene variants are associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
Ioannis Zaganas, Ioannis Tsiverdis, Evgenia Kokosali, Ionela Litso, Minas Drakos, Irene Skoula, Alexandros Zabetakis, Lambros Mathioudakis, Vassilios Mastorodemos, Panayiotis Mitsias   +9 more
doaj   +2 more sources

Microglial reactivity predicts hippocampal, but not global, atrophy in cerebral small vessel disease. [PDF]

Alzheimers Dement
Abstract INTRODUCTION Cerebral small vessel disease (CSVD) is the most prevalent pathology underlying vascular dementia. Increased neuroinflammation and blood‐brain barrier (BBB) permeability have been implicated in CSVD pathogenesis. We determined whether microglial reactivity and BBB permeability at baseline predicted whole‐brain and hippocampal ...
Zainurin A, Brown RB, Tozer DJ, Markus HS.   +3 more
europepmc   +2 more sources

Vascular smooth muscle cell loss, but not neuroinflammation, drives cerebrovascular reactivity impairment in Alzheimer's disease. [PDF]

Alzheimers Dement
Abstract INTRODUCTION Cerebrovascular reactivity (CVR) impairment is a key feature of Alzheimer's disease and related dementias (ADRD), but its mechanistic basis remains unclear. This study examined whether vascular smooth muscle cell (VSMC) loss, rather than amyloidosis or neuroinflammation, underlies CVR deficits.
Yang X, Li Y, Yao M, Bibic A, Duan W, Lu H, Wei Z.   +6 more
europepmc   +2 more sources

Induced pluripotent stem cell model revealed impaired neurovascular interaction in genetic small vessel disease Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Frontiers in Cellular Neuroscience, 2023
IntroductionCerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is the most common genetic small vessel disease caused by variants in the NOTCH3 gene.
Wenjun Zhang, Xiangjun Zhao, Xuewei Qi, Susan J. Kimber, Nigel M. Hooper, Nigel M. Hooper, Tao Wang, Tao Wang, Tao Wang   +8 more
doaj   +1 more source

Neuroimaging Characteristics of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) in Korean Based on Jeju Cohort: A Pictorial Essay

Journal of the Korean Society of Radiology, 2023
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small artery vasculopathy caused by mutations in the NOTCH3 gene on chromosome 19.
Yeh Rin Suh, Ho Kyu Lee, Kyeong Ho Jung, Jung Seok Lee, Jay Chol Choi   +4 more
doaj   +1 more source

Human iPS cell-derived mural cells as an in vitro model of hereditary cerebral small vessel disease

Molecular Brain, 2020
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of the most common forms of hereditary cerebral small vessel diseases and is caused by mutations in NOTCH3.
Yumi Yamamoto, Katsutoshi Kojima, Daisuke Taura, Masakatsu Sone, Kazuo Washida, Naohiro Egawa, Takayuki Kondo, Eiko N. Minakawa, Kayoko Tsukita, Takako Enami, Hidekazu Tomimoto, Toshiki Mizuno, Raj N. Kalaria, Nobuya Inagaki, Ryosuke Takahashi, Mariko Harada-Shiba, Masafumi Ihara, Haruhisa Inoue   +17 more
doaj   +1 more source

Headache and NOTCH3 Gene Variants in Patients with CADASIL

Neurology International, 2023
Autosomal dominant cerebral arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited vascular disease characterized by recurrent strokes, cognitive impairment, psychiatric symptoms, apathy, and migraine.
Oliwia Szymanowicz, Izabela Korczowska-Łącka, Bartosz Słowikowski, Małgorzata Wiszniewska, Ada Piotrowska, Ulyana Goutor, Paweł P. Jagodziński, Wojciech Kozubski, Jolanta Dorszewska   +8 more
doaj   +1 more source