Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis
Abstract Background Niemann-Pick disease Type C1 (NPC1) is a rare progressive neurodegenerative disorder caused by mutations in the NPC1 gene. The pathological mechanisms, underlying NPC1 are not yet completely understood. Especially the contribution of glial cells and gliosis to the progression of...
Main Authors: | Franziska Peter, Sebastian Rost, Arndt Rolfs, Moritz J. Frech |
---|---|
Format: | Article |
Language: | English |
Published: |
BMC
2017-08-01
|
Series: | Orphanet Journal of Rare Diseases |
Subjects: | |
Online Access: | http://link.springer.com/article/10.1186/s13023-017-0697-y |
Similar Items
-
Glial fibrillary acidic protein (GFAP): modulation by growth factors and its implication in astrocyte differentiation
by: F.C.A. Gomes, et al.
Published: (1999-05-01) -
Sacsin Deletion Induces Aggregation of Glial Intermediate Filaments
by: Fernanda Murtinheira, et al.
Published: (2022-01-01) -
Oxidative Stress and Alterations in the Antioxidative Defense System in Neuronal Cells Derived from NPC1 Patient-Specific Induced Pluripotent Stem Cells
by: Alexandra V. Jürs, et al.
Published: (2020-10-01) -
Patient-Specific iPSC-Derived Neural Differentiated and Hepatocyte-like Cells, Carrying the Compound Heterozygous Mutation p.V1023Sfs*15/p.G992R, Present the “Variant” Biochemical Phenotype of Niemann-Pick Type C1 Disease
by: Christin Völkner, et al.
Published: (2021-11-01) -
Pluripotent Stem Cells for Disease Modeling and Drug Discovery in Niemann-Pick Type C1
by: Christin Völkner, et al.
Published: (2021-01-01)