Late onset Tay–Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system

2004 ◽  
Vol 1001 (1-2) ◽  
pp. 37-50 ◽  
Author(s):  
Elena I Miklyaeva ◽  
Weijia Dong ◽  
Alexandre Bureau ◽  
Roya Fattahie ◽  
Yongqin Xu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Late Onset ◽  
Behavioral Changes ◽  
Targeted Disruption ◽  
Tay Sachs Disease ◽  
Hexa Gene ◽  
The Central Nervous System

scholarly journals Conditional Mutation of Rb Causes Cell Cycle Defects without Apoptosis in the Central Nervous System

2003 ◽  
Vol 23 (3) ◽  
pp. 1044-1053 ◽  
Author(s):  
D. MacPherson ◽  
J. Sage ◽  
D. Crowley ◽  
A. Trumpp ◽  
R. T. Bronson ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Central Nervous System ◽  
Nervous System ◽  
Muscle Development ◽  
Brain Size ◽  
Targeted Disruption ◽  
Show Evidence ◽  
The Central Nervous System ◽  
P53 Activation ◽  
Phase Entry

ABSTRACT Targeted disruption of the retinoblastoma gene in mice leads to embryonic lethality in midgestation accompanied by defective erythropoiesis. Rb −/− embryos also exhibit inappropriate cell cycle activity and apoptosis in the central nervous system (CNS), peripheral nervous system (PNS), and ocular lens. Loss of p53 can prevent the apoptosis in the CNS and lens; however, the specific signals leading to p53 activation have not been determined. Here we test the hypothesis that hypoxia caused by defective erythropoiesis in Rb-null embryos contributes to p53-dependent apoptosis. We show evidence of hypoxia in CNS tissue from Rb −/− embryos. The Cre-loxP system was then used to generate embryos in which Rb was deleted in the CNS, PNS and lens, in the presence of normal erythropoiesis. In contrast to the massive CNS apoptosis in Rb-null embryos at embryonic day 13.5 (E13.5), conditional mutants did not have elevated apoptosis in this tissue. There was still significant apoptosis in the PNS and lens, however. Rb −/− cells in the CNS, PNS, and lens underwent inappropriate S-phase entry in the conditional mutants at E13.5. By E18.5, conditional mutants had increased brain size and weight as well as defects in skeletal muscle development. These data support a model in which hypoxia is a necessary cofactor in the death of CNS neurons in the developing Rb mutant embryo.

Pathologic and Behavioral Changes in Mice after Deuteron Irradiation of the Central Nervous System

1963 ◽  
Vol 18 (1) ◽  
pp. 31 ◽  
Author(s):  
J. M. Ordy ◽  
H. W. Barnes ◽  
T. Samorajski ◽  
H. J. Curtis ◽  
L. Wolin ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Behavioral Changes ◽  
Deuteron Irradiation ◽  
The Central Nervous System

scholarly journals Pathophysiology of Bacterial Infection of the Central Nervous System and its Putative Role in the Pathogenesis of Behavioral Changes

2013 ◽  
Vol 35 (1) ◽  
pp. 81-87 ◽  
Author(s):  
Tatiana Barichello ◽  
Jaqueline S. Generoso ◽  
Graziele Milioli ◽  
Samuel G. Elias ◽  
Antônio Lúcio Teixeira
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Bacterial Infection ◽  
Behavioral Changes ◽  
Putative Role ◽  
The Central Nervous System

Mutations resulting in transient and localized degeneration in the developing zebrafish brain

1997 ◽  
Vol 75 (5) ◽  
pp. 579-600 ◽  
Author(s):  
Michael Rodriguez ◽  
Wolfgang Driever
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Large Scale ◽  
Late Onset ◽  
Pharyngeal Arches ◽  
Pectoral Fins ◽  
Group 4 ◽  
Entry Points ◽  
The Central Nervous System ◽  
Group 2

In a large-scale mutagenesis screen in the zebrafish, Danio rerio, we have identified a heterogeneous group of 30 recessive, embryonic lethal mutations characterized by degeneration in the developing central nervous system that is either transient or initially localized to one area of the brain. Transient degeneration is defined as abnormal cell death occurring during a restricted period of development. Following degeneration, the affected structures do not appear to regenerate. In each case degeneration is identified after somitogenesis is complete and is not associated with visually identified patterning defects. These 30 mutations, forming 21 complementation groups, have been classified into four phenotypic groups: group 1, transient degeneration (13 mutations); group 2, spreading degeneration, early onset, in which degeneration is initially confined to the optic tectum but subsequently spreads to other areas of the central nervous system (7 mutations); group 3, late-onset degeneration, initially identified after 4 days (6 mutations); and group 4, degeneration with abnormal pigmentation (4 mutations). Although apoptotic cells are seen in the retina and tectum of all mutants, the distribution, temporal progression, and severity of degeneration vary between mutations. Several mutations also show pleiotropic effects, with degeneration involving extraneural structures including the pharyngeal arches and pectoral fins. We discuss some of the pathways important for cell survival in the nervous system and suggest that these mutations will provide entry points for identifying genes that affect the survival of restricted neural populations.

scholarly journals Toxic Effects of Mercury on the Cardiovascular and Central Nervous Systems

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Bruna Fernandes Azevedo ◽  
Lorena Barros Furieri ◽  
Franck Maciel Peçanha ◽  
Giulia Alessandra Wiggers ◽  
Paula Frizera Vassallo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Endothelial Function ◽  
Environmental Contamination ◽  
Human Activities ◽  
Behavioral Changes ◽  
Low Doses ◽  
High Exposure ◽  
Central Nervous Systems ◽  
The Central Nervous System

Environmental contamination has exposed humans to various metal agents, including mercury. This exposure is more common than expected, and the health consequences of such exposure remain unclear. For many years, mercury was used in a wide variety of human activities, and now, exposure to this metal from both natural and artificial sources is significantly increasing. Many studies show that high exposure to mercury induces changes in the central nervous system, potentially resulting in irritability, fatigue, behavioral changes, tremors, headaches, hearing and cognitive loss, dysarthria, incoordination, hallucinations, and death. In the cardiovascular system, mercury induces hypertension in humans and animals that has wide-ranging consequences, including alterations in endothelial function. The results described in this paper indicate that mercury exposure, even at low doses, affects endothelial and cardiovascular function. As a result, the reference values defining the limits for the absence of danger should be reduced.

scholarly journals Late Onset of Cerebellar Abiotrophy in a Boxer Dog

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Sanjeev Gumber ◽  
Doo-Youn Cho ◽  
Timothy W. Morgan
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Immunohistochemical Staining ◽  
Late Onset ◽  
Histopathological Examination ◽  
Head Tilt ◽  
Neurological Signs ◽  
History Of ◽  
The Central Nervous System ◽  
Degenerative Disorder

Cerebellar abiotrophy is a degenerative disorder of the central nervous system and has been reported in humans and animals. This case report documents clinical, histopathological, and immunohistochemical findings of cerebellar abiotrophy in an adult Boxer dog. A 3.5-year-old, female, tan Boxer dog presented with a six-week history of left-sided head tilt. Neurological examination and additional diagnostics during her three subsequent visits over 4.5 months revealed worsening of neurological signs including marked head pressing, severe proprioceptive deficits in all the four limbs, loss of menace response and palpebral reflex in the left eye, and a gradual seizure lasting one hour at her last visit. Based on the immunohistochemical staining for glial fibrillary acidic protein and histopathological examination of cerebellum, cerebellar cortical abiotrophy was diagnosed. This is the first reported case of cerebellar abiotrophy in a Boxer dog to our knowledge.

scholarly journals Behavioral Effects of Gangliosides: Anatomical Considerations

1992 ◽  
Vol 3 (1) ◽  
pp. 1-19 ◽  
Author(s):  
Dwaine F. Emerich
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Loss ◽  
Behavioral Changes ◽  
Pharmacological Treatments ◽  
Behavioral Recovery ◽  
The Central Nervous System ◽  
Developing Neurons ◽  
Over 40 Years ◽  
Enhance Cell Survival

Gangliosides are endogenous sialic acid containing glycospingolipids which are highly concentrated in the central nervous system. Although they were first characterized over 40 years ago, the function(s) played by this unique class of lipids remain largely unknown. Gangliosides have been suggested to play a prominent role in both normal and abnormal developmental processes. In addition, several lines of convergent evidence have indicated that gangliosides exert pronounced trophic effects following damage to peripheral and central nerves. Gangliosides have been shown to (1) enhance cell survival and outgrowth in cultured and developing neurons; (2) promote the regeneration of damaged peripheral and central nerves, and (3) facilitate behavioral recovery by altering the pattern, extent and persistence of the biochemical, morphological and behavioral changes induced by neural trauma. Little is known, however, concerning the neurobiological mechanisms which subserve the. behavioral protection afforded by ganglioside treatment. This review focuses on the evidence suggesting that gangliosides mediate functional recovery by minimizing primary or secondary cell loss or promoting the regeneration or sprouting of damaged central nerves subsequent to injury. An understanding of the mechanisms, by which gangliosides produce their effects may lead to the development of more efficacious and rational primary or adjunct pharmacological treatments for central nervous system disorders.

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