scholarly journals Neurovascular dysfunction and neuroinflammation in a Cockayne syndrome mouse model

Aging ◽  
2021 ◽  
Author(s):  
Gustavo Satoru Kajitani ◽  
Lear Brace ◽  
Jose Humberto Trevino-Villarreal ◽  
Kaspar Trocha ◽  
Michael Robert MacArthur ◽  
...  
Keyword(s):  
Mouse Model ◽  
Cockayne Syndrome ◽  
Neurovascular Dysfunction

scholarly journals An Xpd mouse model for the combined xeroderma pigmentosum/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria

Cancer Cell ◽  
2006 ◽  
Vol 10 (2) ◽  
pp. 121-132 ◽  
Author(s):  
Jaan-Olle Andressoo ◽  
James R. Mitchell ◽  
Jan de Wit ◽  
Deborah Hoogstraten ◽  
Marcel Volker ◽  
...  
Keyword(s):  
Mouse Model ◽  
Xeroderma Pigmentosum ◽  
Cockayne Syndrome ◽  
Cancer Predisposition

P3-464: CARBONIC ANHYDRASE INHIBITORS AMELIORATE NEUROVASCULAR DYSFUNCTION IN A MOUSE MODEL OF CEREBRAL AMYLOID ANGIOPATHY

2006 ◽  
Vol 14 (7S_Part_24) ◽  
pp. P1296-P1296 ◽  
Author(s):  
Federica Angiulli ◽  
Maria E. Solesio ◽  
Ludovic Debure ◽  
Jaime Ramos Cejudo ◽  
Thomas Wisniewski ◽  
...  
Keyword(s):  
Carbonic Anhydrase ◽  
Mouse Model ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Carbonic Anhydrase Inhibitors ◽  
Cerebral Amyloid ◽  
Neurovascular Dysfunction

Dendritic spine density is altered in a mouse model of Cockayne syndrome

2013 ◽  
Vol 39 (4) ◽  
pp. 437-440 ◽  
Author(s):  
J. C. Wong ◽  
N. P. Visanji ◽  
M. K. Dabek ◽  
R. R. Laposa ◽  
L.-N. Hazrati
Keyword(s):  
Mouse Model ◽  
Dendritic Spine ◽  
Cockayne Syndrome ◽  
Spine Density ◽  
Dendritic Spine Density

scholarly journals Age-Dependent Neurovascular Dysfunction and Damage in a Mouse Model of Cerebral Amyloid Angiopathy

Stroke ◽  
2014 ◽  
Vol 45 (6) ◽  
pp. 1815-1821 ◽  
Author(s):  
Laibaik Park ◽  
Kenzo Koizumi ◽  
Sleiman El Jamal ◽  
Ping Zhou ◽  
Mary Lou Previti ◽  
...  
Keyword(s):  
Mouse Model ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Age Dependent ◽  
Cerebral Amyloid ◽  
Neurovascular Dysfunction

scholarly journals Lifespan extension by dietary intervention in a mouse model of Cockayne Syndrome uncouples early postnatal development from segmental progeria

Aging Cell ◽  
2013 ◽  
Vol 12 (6) ◽  
pp. 1144-1147 ◽  
Author(s):  
Lear E. Brace ◽  
Sarah C. Vose ◽  
Dorathy F. Vargas ◽  
Shuangyun Zhao ◽  
Xiu-Ping Wang ◽  
...  
Keyword(s):  
Mouse Model ◽  
Postnatal Development ◽  
Dietary Intervention ◽  
Cockayne Syndrome ◽  
Lifespan Extension ◽  
Early Postnatal Development

HDAC inhibition improves autophagic and lysosomal function to prevent loss of subcutaneous fat in a mouse model of Cockayne syndrome

2018 ◽  
Vol 10 (456) ◽  
pp. eaam7510 ◽  
Author(s):  
Marc Majora ◽  
Kevin Sondenheimer ◽  
Maren Knechten ◽  
Ingo Uthe ◽  
Charlotte Esser ◽  
...  
Keyword(s):  
Mouse Model ◽  
Subcutaneous Fat ◽  
Cockayne Syndrome ◽  
Hdac Inhibition ◽  
Lysosomal Function

scholarly journals Retinal Degeneration and Ionizing Radiation Hypersensitivity in a Mouse Model for Cockayne Syndrome

2006 ◽  
Vol 27 (4) ◽  
pp. 1433-1441 ◽  
Author(s):  
Theo G. M. F. Gorgels ◽  
Ingrid van der Pluijm ◽  
Renata M. C. Brandt ◽  
George A. Garinis ◽  
Harry van Steeg ◽  
...  
Keyword(s):  
Ionizing Radiation ◽  
Mouse Model ◽  
Photoreceptor Cells ◽  
Cockayne Syndrome ◽  
Dna Lesions ◽  
Premature Aging ◽  
Oxidative Stress Marker ◽  
Marker Genes ◽  
Uv Sensitivity ◽  
Transcription Coupled Repair

ABSTRACT Mutations in the CSB gene cause Cockayne syndrome (CS), a DNA repair disorder characterized by UV sensitivity and severe physical and neurological impairment. CSB functions in the transcription-coupled repair subpathway of nucleotide excision repair. This function may explain the UV sensitivity but hardly clarifies the other CS symptoms. Many of these, including retinopathy, are associated with premature aging. We studied eye pathology in a mouse model for CS. Csb m/m mice were hypersensitive to UV light and developed epithelial hyperplasia and squamous cell carcinomas in the cornea, which underscores the importance of transcription-coupled repair of photolesions in the mouse. In addition, we observed a spontaneous loss of retinal photoreceptor cells with age in the Csb m/m retina, resulting in a 60% decrease in the number of rods by the age of 18 months. Importantly, when Csb m/m mice (as well as Csa −/− mice) were exposed to 10 Gy of ionizing radiation, we noticed an increase in apoptotic photoreceptor cells, which was not observed in wild-type animals. This finding, together with our observation that the expression of established oxidative stress marker genes is upregulated in the Csb m/m retina, suggests that (endogenous) oxidative DNA lesions play a role in this CS-specific premature-aging feature and supports the oxidative DNA damage theory of aging.

Regenerating myofibers in tibialis anterior muscles of young ‘MDX’ mice

1987 ◽  
Vol 45 ◽  
pp. 726-727
Author(s):  
H. D. Geissinge ◽  
L.D. Rhodes
Keyword(s):  
Muscular Dystrophy ◽  
Mouse Model ◽  
Tibialis Anterior ◽  
Physiological Activity ◽  
Mdx Mice ◽  
Mode Of Inheritance

A recently discovered mouse model (‘mdx’) for muscular dystrophy in man may be of considerable interest, since the disease in ‘mdx’ mice is inherited by the same mode of inheritance (X-linked) as the human Duchenne (DMD) muscular dystrophy. Unlike DMD, which results in a situation in which the continual muscle destruction cannot keep up with abortive regenerative attempts of the musculature, and the sufferers of the disease die early, the disease in ‘mdx’ mice appears to be transient, and the mice do not die as a result of it. In fact, it has been reported that the severely damaged Tibialis anterior (TA) muscles of ‘mdx’ mice seem to display exceptionally good regenerative powers at 4-6 weeks, so much so, that these muscles are able to regenerate spontaneously up to their previous levels of physiological activity.

Mouse model of gastric infection with cytotoxin-expressing strain of Helicobacter pylori in studying of pathogenesis of chronic gastric ulcer

1998 ◽  
Vol 13 (11-s4) ◽  
pp. S178-S184 ◽  
Author(s):  
PETER KONTUREK ◽  
TOMASZ BRZOZOWSKI ◽  
STANISLAW KONTUREK ◽  
ELZBIETA KARCZEWSKA ◽  
ROBERT PAJDO ◽  
...  
Keyword(s):  
Helicobacter Pylori ◽  
Gastric Ulcer ◽  
Mouse Model ◽  
Chronic Gastric Ulcer
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