Cargando…
Caenorhabditis elegans dnj-14, the orthologue of the DNAJC5 gene mutated in adult onset neuronal ceroid lipofuscinosis, provides a new platform for neuroprotective drug screening and identifies a SIR-2.1-independent action of resveratrol
Adult onset neuronal lipofuscinosis (ANCL) is a human neurodegenerative disorder characterized by progressive neuronal dysfunction and premature death. Recently, the mutations that cause ANCL were mapped to the DNAJC5 gene, which encodes cysteine string protein alpha. We show here that mutating dnj-...
Autores principales: | Kashyap, Sudhanva S., Johnson, James R., McCue, Hannah V., Chen, Xi, Edmonds, Matthew J., Ayala, Mimieveshiofuo, Graham, Margaret E., Jenn, Robert C., Barclay, Jeff W., Burgoyne, Robert D., Morgan, Alan |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2014
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4204773/ https://www.ncbi.nlm.nih.gov/pubmed/24947438 http://dx.doi.org/10.1093/hmg/ddu316 |
Ejemplares similares
-
Expression profile of a Caenorhabditis elegans model of adult neuronal ceroid lipofuscinosis reveals down regulation of ubiquitin E3 ligase components
por: McCue, Hannah V., et al.
Publicado: (2015) -
Exome-Sequencing Confirms DNAJC5 Mutations as Cause of Adult Neuronal Ceroid-Lipofuscinosis
por: Benitez, Bruno A., et al.
Publicado: (2011) -
Adult-Onset Neuronal Ceroid Lipofuscinosis With a Novel DNAJC5 Mutation Exhibits Aberrant Protein Palmitoylation
por: Huang, Qiang, et al.
Publicado: (2022) -
Abnormal triaging of misfolded proteins by adult neuronal ceroid lipofuscinosis-associated DNAJC5/CSPα mutants causes lipofuscin accumulation
por: Lee, Juhyung, et al.
Publicado: (2022) -
MRI findings in neuronal ceroid lipofuscinosis
por: Crain, Anna M., et al.
Publicado: (2020)