Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis
Author:
Publisher
Springer Science and Business Media LLC
Subject
Pharmacology (medical),Genetics(clinical),General Medicine
Link
http://link.springer.com/content/pdf/10.1186/s13023-017-0697-y.pdf
Reference65 articles.
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2. Vanier MT. Niemann-pick disease type C. Orphanet J Rare Dis. 2010;5:16. doi: 10.1186/1750-1172-5-16 .
3. Zervas M, Dobrenis K, Walkley SU. Neurons in Niemann-pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. J Neuropathol Exp Neurol. 2001;60:49–64.
4. Infante RE, Wang ML, Radhakrishnan A, Kwon HJ, Brown MS, Goldstein JL. NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci U S A. 2008;105:15287–92. doi: 10.1073/pnas.0807328105 .
5. Giese A-K, Mascher H, Grittner U, Eichler S, Kramp G, Lukas J, et al. A novel, highly sensitive and specific biomarker for Niemann-pick type C1 disease. Orphanet J Rare Dis. 2015;10:2006. doi: 10.1186/s13023-015-0274-1 .
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