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DA strain of Theiler's murine encephalomyelitis virus (TMEV) persists in the mouse central nervous system (CNS) and induces demyelination while GDVII strain fails to persist or demyelinate. L* protein, which is synthesized only in DA but not GDVII, is believed important in virus persistence and demyelination. Because a major reservoir for DA persistence is infiltrated macrophages or microglia, a resident...
Sandhoff disease is a lysosomal storage disorder characterized by the absence of β-hexosaminidase and storage of G M2 ganglioside and related glycolipids in the central nervous system. The glycolipid storage causes severe neurodegeneration and progressive decline of neurological function that leads to death at an early stage of life. The pathogenetic mechanisms of neurodegeneration in...
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