Demyelinating Diseases

Publication Title: 
Human Molecular Genetics

Niemann-Pick type C (NPC) disease, an autosomal recessive disorder caused primarily by loss-of-function mutations in NPC1 gene, is characterized neuropathologically by intracellular cholesterol accumulation, gliosis and neuronal loss in selected brain regions. Recent studies have shown that NPC disease exhibits intriguing parallels with Alzheimer's disease (AD), including the presence of tau-positive neurofibrillary tangles (NFTs) and ?-amyloid (A?)-related peptides in vulnerable brain regions. Since enhanced cholesterol level, which acts as a risk factor for AD, can increase A?

Author(s): 
Maulik, Mahua
Ghoshal, Bibaswan
Kim, John
Wang, Yanlin
Yang, Jing
Westaway, David
Kar, Satyabrata
Publication Title: 
Glia

Steroidal estrogens can regulate inflammatory immune responses and may be involved in the suppression of multiple sclerosis (MS) during pregnancy. However, the risks and side effects associated with steroidal estrogens may limit their usefulness for long-term MS therapy. Selective estrogen receptor modulators (SERMs) could provide an alternative therapeutic strategy, because they behave as estrogen agonists in some tissues, but are either inert or behave like estrogen antagonists in other tissues.

Author(s): 
Bebo, Bruce F.
Dehghani, Babak
Foster, Scott
Kurniawan, Astrid
Lopez, Francisco J.
Sherman, Larry S.
Publication Title: 
The Journal of Biological Chemistry

An increase in CNS remyelination and a decrease in CNS inflammation are important steps to halt the progression of multiple sclerosis. Earlier studies have shown that gemfibrozil, a lipid-lowering drug, has anti-inflammatory properties.

Author(s): 
Jana, Malabendu
Mondal, Susanta
Gonzalez, Frank J.
Pahan, Kalipada
Publication Title: 
The Journal of Neuroscience: The Official Journal of the Society for Neuroscience

The Long-Evans shaker (les) rat has a mutation in myelin basic protein that results in severe CNS dysmyelination and subsequent demyelination during development. During this time, les oligodendrocytes accumulate cytoplasmic vesicles, including lysosomes and membrane-bound organelles. However, the mechanism and functional relevance behind these oligodendrocyte abnormalities in les have not been investigated. Using high-magnification electron microscopy, we identified the accumulations in les oligodendrocytes as early and late autophagosomes.

Author(s): 
Smith, Chelsey M.
Mayer, Joshua A.
Duncan, Ian D.
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