Cargando…
Skeletal muscle cell protein dysregulation highlights the pathogenesis mechanism of myopathy-associated p97/VCP R155H mutations
p97/VCP, a hexametric member of the AAA-ATPase superfamily, has been associated with a wide range of cellular protein pathways, such as proteasomal degradation, the unfolding of polyubiquitinated proteins, and autophagosome maturation. Autosomal dominant p97/VCP mutations cause a rare hereditary mul...
Autores principales: | Luzzi, Anna, Wang, Feng, Li, Shan, Iacovino, Michelina, Chou, Tsui-Fen |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435852/ https://www.ncbi.nlm.nih.gov/pubmed/37602234 http://dx.doi.org/10.3389/fneur.2023.1211635 |
Ejemplares similares
-
VCP/p97 inhibitor CB-5083 modulates muscle pathology in a mouse model of VCP inclusion body myopathy
por: Cheng, Cheng, et al.
Publicado: (2022) -
Specific mutations in the D1–D2 linker region of VCP/p97 enhance ATPase activity and confer resistance to VCP inhibitors
por: Bastola, Prabhakar, et al.
Publicado: (2017) -
A structure- and chemical genomics-based approach for repositioning of drugs against VCP/p97 ATPase
por: Segura-Cabrera, Aldo, et al.
Publicado: (2017) -
Rare Manifestation of a c.290 C>T, p.Gly97Glu VCP Mutation
por: Jerath, Nivedita U., et al.
Publicado: (2015) -
Anti–Valosin‐Containing Protein (VCP/p97) Autoantibodies in Inclusion Body Myositis and Other Inflammatory Myopathies
por: Amlani, Adam, et al.
Publicado: (2022)