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content:aicardi-goutieres_syndrome [2020/02/02 14:14] – [Diagnosis] icna | content:aicardi-goutieres_syndrome [2020/02/21 07:30] – [References] bijuhameed | ||
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Both AGS and many placentally acquired viral infections are characterized by the production of high levels of IFNα. Astrocyte-specific chronic overproduction of IFNα in transgenic mice has been shown to recapitulate the neuropathologic findings seen in AGS[(: | Both AGS and many placentally acquired viral infections are characterized by the production of high levels of IFNα. Astrocyte-specific chronic overproduction of IFNα in transgenic mice has been shown to recapitulate the neuropathologic findings seen in AGS[(: | ||
- | The actual pathomechanisms that lead to CNS damage in AGS patients are unknown. In a mouse model transgenic for a glial fibrillary acidic protein-interferon-alpha fusion protein where IFNα is expressed in an astrocyte-restricted fashion, transgenic animals develop a progressive inflammatory encephalopathy with neuropathologic features similar to AGS [66] suggesting a neurotoxic role of IFNα in the developing brain[(: | + | The actual pathomechanisms that lead to CNS damage in AGS patients are unknown. In a mouse model transgenic for a glial fibrillary acidic protein-interferon-alpha fusion protein |
Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses[(: | Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses[(: | ||
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~~REFNOTES cite~~ | ~~REFNOTES cite~~ | ||
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