Cargando…

Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity

The neuronal membrane-associated periodic spectrin skeleton (MPS) contributes to neuronal development, remodeling, and organization. Post-translational modifications impinge on spectrin, the major component of the MPS, but their role remains poorly understood. One modification targeting spectrin is...

Descripción completa

Detalles Bibliográficos
Autores principales:	Miazek, Arkadiusz, Zalas, Michał, Skrzymowska, Joanna, Bogin, Bryan A., Grzymajło, Krzysztof, Goszczynski, Tomasz M., Levine, Zachary A., Morrow, Jon S., Stankewich, Michael C.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2021
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8012654/ https://www.ncbi.nlm.nih.gov/pubmed/33790315 http://dx.doi.org/10.1038/s41598-021-86470-1

Ejemplares similares

Publisher Correction: Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity
por: Miazek, Arkadiusz, et al.
Publicado: (2021)

Progressive Ataxia, Memory Impairments, and Seizure Episodes in Spna2 R1098Q Mouse Variant Affecting Alpha II Spectrin’s Scaffold Stability
por: Zalas, Michał, et al.
Publicado: (2023)

The Spread of Spectrin in Ataxia and Neurodegenerative Disease
por: Morrow, Jon S., et al.
Publicado: (2021)

Isoforms of Spectrin and Ankyrin Reflect the Functional Topography of the Mouse Kidney
por: Stankewich, Michael C., et al.
Publicado: (2016)

Inhibition of calpain but not caspase activity by spectrin fragments
por: Rolius, Ramunas, et al.
Publicado: (2010)

Expanding the β-III Spectrin-Associated Phenotypes toward Non-Progressive Congenital Ataxias with Neurodegeneration
por: Sancho, Paula, et al.
Publicado: (2021)

Spectrin mutations that cause spinocerebellar ataxia type 5 impair axonal transport and induce neurodegeneration in Drosophila
por: Lorenzo, Damaris N., et al.
Publicado: (2010)

Neuroprotective strategies against calpain-mediated neurodegeneration
por: Yildiz-Unal, Aysegul, et al.
Publicado: (2015)

Developmental Changes in Expression of βIV Spectrin Splice Variants at Axon Initial Segments and Nodes of Ranvier
por: Yoshimura, Takeshi, et al.
Publicado: (2017)

Nodal β spectrins are required to maintain Na(+) channel clustering and axon integrity
por: Liu, Cheng-Hsin, et al.
Publicado: (2020)

β spectrin-dependent and domain specific mechanisms for Na(+) channel clustering
por: Liu, Cheng-Hsin, et al.
Publicado: (2020)

Cerebellar ataxias: β‐III spectrin's interactions suggest common pathogenic pathways
por: Perkins, Emma, et al.
Publicado: (2016)

Retraction: Sialoglycosylation of RBC in Visceral Leishmaniasis Leads to Enhanced Oxidative Stress, Calpain-Induced Fragmentation of Spectrin and Hemolysis
Publicado: (2019)

A HIERARCHY OF ANKYRIN/SPECTRIN COMPLEXES CLUSTERS SODIUM CHANNELS AT NODES OF RANVIER
por: Ho, Tammy Szu-Yu, et al.
Publicado: (2014)

Calpains as novel players in the molecular pathogenesis of spinocerebellar ataxia type 17
por: Weber, Jonasz Jeremiasz, et al.
Publicado: (2022)

Novel drug discovery platform for spinocerebellar ataxia, using fluorescence technology targeting β-III-spectrin
por: Rebbeck, Robyn T., et al.
Publicado: (2020)

Spectrin is peripheral
por: Bonetta, Laura
Publicado: (2005)

Novel Bioinformatics–Based Approach for Proteomic Biomarkers Prediction of Calpain-2 & Caspase-3 Protease Fragmentation: Application to βII-Spectrin Protein
por: El-Assaad, Atlal, et al.
Publicado: (2017)

Plasma Markers of Neurodegeneration Are Raised in Friedreich’s Ataxia
por: Zeitlberger, Anna M., et al.
Publicado: (2018)

The time dimension of neurodegeneration: the example of Friedreich’s ataxia
por: Vannocci, Tommaso, et al.
Publicado: (2017)

Increased actin binding is a shared molecular consequence of numerous spinocerebellar ataxia mutations in β-III-spectrin
por: Atang, Alexandra E., et al.
Publicado: (2023)

Spectrin functions upstream of ankyrin in a spectrin cytoskeleton assembly pathway
por: Das, Amlan, et al.
Publicado: (2006)

Avian lens spectrin: subunit composition compared with erythrocyte and brain spectrin
Publicado: (1983)

Non-erythroid beta spectrin interacting proteins and their effects on spectrin tetramerization
por: Sevinc, Akin, et al.
Publicado: (2011)

Calpains in cyanobacteria and the origin of calpains
por: Vešelényiová, Dominika, et al.
Publicado: (2022)

Role of Spectrin in Endocytosis
por: Li, Donghai
Publicado: (2022)

A Novel Missense Mutation in the Spectrin Beta Nonerythrocytic 2 Gene Likely Associated with Spinocerebellar Ataxia Type 5
por: Liu, Li-Zhi, et al.
Publicado: (2016)

Early-phase drug discovery of β-III-spectrin actin-binding modulators for treatment of spinocerebellar ataxia type 5
por: Guhathakurta, Piyali, et al.
Publicado: (2023)

Golgi spectrin: identification of an erythroid beta-spectrin homolog associated with the Golgi complex
Publicado: (1994)

Beta spectrin bestows protein 4.1 sensitivity on spectrin-actin interactions
Publicado: (1987)

In vivo assessment of neurodegeneration in Spinocerebellar Ataxia type 7
por: Parker, Jacob A., et al.
Publicado: (2021)

Reply to: “Microvascular Breakdown Due to Retinal Neurodegeneration in Ataxias”
por: Turski, Christopher A., et al.
Publicado: (2022)

Sphingosine-1-phosphate links glycosphingolipid metabolism to neurodegeneration via a calpain-mediated mechanism
por: Hagen, N, et al.
Publicado: (2011)

αI-spectrin represents evolutionary optimization of spectrin for red blood cell deformability
por: Hale, John, et al.
Publicado: (2021)

A human β-III-spectrin spinocerebellar ataxia type 5 mutation causes high-affinity F-actin binding
por: Avery, Adam W., et al.
Publicado: (2016)

Neural cell adhesion molecule (NCAM) association with PKCβ(2) via βI spectrin is implicated in NCAM-mediated neurite outgrowth
por: Leshchyns'ka, Iryna, et al.
Publicado: (2003)

Brain spectrin(240/235) and brain spectrin(240/235E): two distinct spectrin subtypes with different locations within mammalian neural cells
Publicado: (1986)

Neurodegeneration in Friedreich's Ataxia: From Defective Frataxin to Oxidative Stress
por: Gomes, Cláudio M., et al.
Publicado: (2013)

Elongator mutation in mice induces neurodegeneration and ataxia-like behavior
por: Kojic, Marija, et al.
Publicado: (2018)

Neurofilament Light Chain Is a Biomarker of Neurodegeneration in Ataxia Telangiectasia
por: Donath, H., et al.
Publicado: (2021)

Cannot write session to /tmp/vufind_sessions/sess_oen29k77nsvffup8l3mhv9h5id