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Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation
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نویسنده
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kyrkanides s. ,brouxhon s.m. ,tallents r.h. ,miller j.-n.h. ,olschowka j.a. ,o'banion m.k.
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منبع
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journal of neuroinflammation - 2012 - دوره : 9 - شماره : 0
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چکیده
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This study evaluated whether gm2 ganglioside storage is necessary for neurodegeneration and neuroinflammation by performing β-hexosaminidase rescue experiments in neurons of hexb-/- mice. we developed a novel mouse model,whereby the expression of the human hexb gene was targeted to neurons of hexb-/- mice by the thy1 promoter. despite β-hexosaminidase restoration in neurons was sufficient in rescuing hexb-/- mice from gm2 neuronal storage and neurodegeneration,brain inflammation persisted,including the presence of large numbers of reactive microglia/macrophages due to persisting gm2 presence in this cell type. in conclusion,our results suggest that neuroinflammation is not sufficient to elicit neurodegeneration as long as neuronal function is restored. © 2012 kyrkanides et al.; licensee biomed central ltd.
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کلیدواژه
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β-hexosaminidase; GM2 gangliosidosis; Mouse; Neuron; Sandhoff disease; Transgenic
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آدرس
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department of children's dentistry,stony brook university,stony brook,ny,11894-8701,united states,health science center,stony brook university,stony brook,ny,11794-8701, United States, health science center,stony brook university,stony brook,ny,11794-8701, United States, department of dentistry,university of rochester school of medicine and dentistry,rochester,ny,14642, United States, department of dentistry,university of rochester school of medicine and dentistry,rochester,ny,14642, United States, neurobiology and anatomy,university of rochester school of medicine and dentistry,rochester,ny,14642, United States, neurobiology and anatomy,university of rochester school of medicine and dentistry,rochester,ny,14642, United States
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Authors
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