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ataxia/necrosis

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Ataxia telangiectasia (AT) is a rare genetic disorder. Symptoms of the disease include cerebellar ataxia, depressed immunoresponsiveness, increased sensitivity to radiation, and leukemias. Various kinds of AT cells show reduced efficiency of differentiation. The ataxia telangiectasia gene (ATM) may
Use of the fluoroquinolone antibiotic trovafloxacin (TVX) was restricted due to idiosyncratic, drug-induced liver injury (IDILI). Previous studies demonstrated that tumor necrosis factor-alpha (TNF) and TVX interact to cause death of hepatocytes in vitro that was associated with prolonged activation
Despite that ataxia-telangiectasia mutated (ATM) is involved in IL-6 promoted lung cancer chemotherapeutic resistance and metastasis, the exact role of ATM in tumor necrosis factor-alpha (TNF-α) increasing tumor migration is still elusive. In the present study, we demonstrated that TNF-α promoted

Ataxia telangiectasia with vascular abnormalities in the brain parenchyma: report of an autopsy case and literature review.

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A 25-year-old man was admitted to the Department of Neurology, Gunma University Hospital, in June 1997. He had an intellectual disability and had suffered from repeated infection since childhood. Cerebellar ataxia had developed at 19 years of age and he had been clinically diagnosed with ataxia

Metaphyeal and Diaphyseal Dysplasia of the Third Cervical Vertebra Secondary to Physeal Necrosis in a Quarter Horse Foal.

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Ischaemia-induced physeal injury has not been described previously in the horse. A 1-month-old Quarter horse foal was submitted for necropsy examination due to an acute onset of ataxia followed by a 4-week history of progressive decline. Focal narrowing of the spinal canal due to ventral compression

[Retrospective analysis of cases with a diagnosis of cerebrocortical necrosis and its relation with type 5 bovine herpesvirus].

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In order to demonstrate the association of bovine herpesvirus type 5 (BHV-5) and cerebrocortical necrosis (CCN), 89 such cases were examined in cattle from three regions of Buenos Aires Province, Argentina, registered between 1970-1999. Hematoxylin-eosin staining and BHV-5 in situ hybridization were

Laminar cortical necrosis in mitochondrial disorders.

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OBJECTIVE Laminar cortical necrosis, defined as focal or diffuse necrosis of one or more cortical lamina, represents an increasingly recognized neuropathological endpoint of vascular, endocrine, immunologic, metabolic, or toxic conditions, of which mitochondrial disorders (MIDs) are the third most

Poliomyelomalacia, pancreatic necrosis, and cerebellar malacia in turkey poults.

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Two-to-5-week-old turkey poults from three large Minnesota flocks exhibited ataxia, flaccid paralysis, and up to 5% mortality as unexpected death. The major post-mortem finding was cerebellar hemorrhage and softening detected in 22 of 89 clinically affected poults. Histologic findings were severe

Regulation of the cell cycle following DNA damage in normal and Ataxia telangiectasia cells.

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A proportion of the population is exposed to acute doses of ionizing radiation through medical treatment or occupational accidents, with little knowledge of the immediate effects. At the cellular level, ionizing radiation leads to the activation of a genetic program which enables the cell to

Loss of MyD88 alters neuroinflammatory response and attenuates early Purkinje cell loss in a spinocerebellar ataxia type 6 mouse model.

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Spinocerebellar ataxia type 6 (SCA6) is dominantly inherited neurodegenerative disease, caused by an expansion of CAG repeat encoding a polyglutamine (PolyQ) tract in the Cav2.1 voltage-gated calcium channel. Its key pathological features include selective degeneration of the cerebellar Purkinje

Silencing of ataxia-telangiectasia mutated by siRNA enhances the in vitro and in vivo radiosensitivity of glioma.

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It is reported that high expression of the ataxia-telangiectasia mutated (ATM) gene is linked with radioresistance in glioma. We hypothesized that the radiosensitivity of this brain tumor is enhanced by silencing of the ATM gene. We transfected the glioma cell line U251 with the siRNA-ATMpuro (group

Ultrastructural observations on spinal ganglion biopsy in Friedreich's ataxia: a preliminary report.

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We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich's ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense
Effective medical treatment and preventive measures for chemical warfare agent sulfur mustard (HD)-caused incapacitating skin toxicity are lacking, because of limited knowledge of its mechanism of action. The proliferating basal epidermal cells are primary major sites of attack during HD-caused skin

Mitochondrial complex I NUBPL mutations cause combined dystonia with bilateral striatal necrosis and cerebellar atrophy.

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The recent advances in genetics have helped to unravel the cause of many dystonia syndromes. With the broadening spectrum of genetically defined dystonia syndromes, distinct clinico-radiological phenotypes are a welcome handle to guide the diagnostic
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