Cargando…
Laforin targets malin to glycogen in Lafora progressive myoclonus epilepsy
Glycogen is the largest cytosolic macromolecule and is kept in solution through a regular system of short branches allowing hydration. This structure was thought to solely require balanced glycogen synthase and branching enzyme activities. Deposition of overlong branched glycogen in the fatal epilep...
Autores principales: | Mitra, Sharmistha, Chen, Baozhi, Wang, Peixiang, Chown, Erin E., Dear, Mathew, Guisso, Dikran R., Mariam, Ummay, Wu, Jun, Gumusgoz, Emrah, Minassian, Berge A. |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9844227/ https://www.ncbi.nlm.nih.gov/pubmed/36511140 http://dx.doi.org/10.1242/dmm.049802 |
Ejemplares similares
-
AAV-Mediated Artificial miRNA Reduces Pathogenic Polyglucosan Bodies and Neuroinflammation in Adult Polyglucosan Body and Lafora Disease Mouse Models
por: Gumusgoz, Emrah, et al.
Publicado: (2022) -
An inducible glycogen synthase-1 knockout halts but does not reverse Lafora disease progression in mice
por: Nitschke, Silvia, et al.
Publicado: (2020) -
Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease
por: Nitschke, Felix, et al.
Publicado: (2017) -
The Laforin–Malin Complex, Involved in Lafora Disease, Promotes the Incorporation of K63-linked Ubiquitin Chains into AMP-activated Protein Kinase β Subunits
por: Moreno, Daniel, et al.
Publicado: (2010) -
Enhanced sensitivity of laforin- and malin-deficient mice to the convulsant agent pentylenetetrazole
por: García-Cabrero, Ana M., et al.
Publicado: (2014)