Cargando…
CoA‐dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases
PKAN, CoPAN, MePAN, and PDH‐E2 deficiency share key phenotypic features but harbor defects in distinct metabolic processes. Selective damage to the globus pallidus occurs in these genetic neurodegenerative diseases, which arise from defects in CoA biosynthesis (PKAN, CoPAN), protein lipoylation (MeP...
Autores principales: | Lambrechts, Roald A, Schepers, Hein, Yu, Yi, van der Zwaag, Marianne, Autio, Kaija J, Vieira‐Lara, Marcel A, Bakker, Barbara M, Tijssen, Marina A, Hayflick, Susan J, Grzeschik, Nicola A, Sibon, Ody CM |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6895606/ https://www.ncbi.nlm.nih.gov/pubmed/31701655 http://dx.doi.org/10.15252/emmm.201910488 |
Ejemplares similares
-
4′‐Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN
por: Jeong, Suh Young, et al.
Publicado: (2019) -
The Role of Acyl-CoA β-Oxidation in Brain Metabolism and Neurodegenerative Diseases
por: Szrok-Jurga, Sylwia, et al.
Publicado: (2023) -
Cofilin/Twinstar Phosphorylation Levels Increase in Response to Impaired Coenzyme A Metabolism
por: Siudeja, Katarzyna, et al.
Publicado: (2012) -
Requirement of the acyl-CoA carrier ACBD6 in myristoylation of proteins: Activation by ligand binding and protein interaction
por: Soupene, Eric, et al.
Publicado: (2020) -
Human VPS13A is associated with multiple organelles and influences mitochondrial morphology and lipid droplet motility
por: Yeshaw, Wondwossen M, et al.
Publicado: (2019)