Cargando…

Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD

Niemann–Pick type C disease is a fatal, progressive neurodegenerative disorder caused by loss-of-function mutations in NPC1, a multipass transmembrane glycoprotein essential for intracellular lipid trafficking. We sought to define the cellular machinery controlling degradation of the most common dis...

Descripción completa

Detalles Bibliográficos
Autores principales:	Schultz, Mark L., Krus, Kelsey L., Kaushik, Susmita, Dang, Derek, Chopra, Ravi, Qi, Ling, Shakkottai, Vikram G., Cuervo, Ana Maria, Lieberman, Andrew P.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2018
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131187/ https://www.ncbi.nlm.nih.gov/pubmed/30202070 http://dx.doi.org/10.1038/s41467-018-06115-2

Ejemplares similares

Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1
por: Chopra, Ravi, et al.
Publicado: (2018)

NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress
por: Höglinger, D., et al.
Publicado: (2019)

Telocytes: a potential defender in the spleen of Npc1 mutant mice
por: Zhang, Bichao, et al.
Publicado: (2016)

ERAD and ERAD tuning: disposal of cargo and of ERAD regulators from the mammalian ER
por: Bernasconi, Riccardo, et al.
Publicado: (2011)

Npc1 Acting in Neurons and Glia Is Essential for the Formation and Maintenance of CNS Myelin
por: Yu, Ting, et al.
Publicado: (2013)

The NPC, in detail
por: Robinson, Richard
Publicado: (2007)

Targeting potassium channels to treat cerebellar ataxia
por: Bushart, David D., et al.
Publicado: (2018)

ERAD and protein import defects in a sec61 mutant lacking ER-lumenal loop 7
por: Tretter, Thomas, et al.
Publicado: (2013)

Identification and Classification of Rare Variants in NPC1 and NPC2 in Quebec
por: Touma, Lahoud, et al.
Publicado: (2021)

Building and breaking the NPC
por: LeBrasseur, Nicole
Publicado: (2003)

2-Hydroxypropyl-gamma-cyclodextrin overcomes NPC1 deficiency by enhancing lysosome-ER association and autophagy
por: Singhal, Ashutosh, et al.
Publicado: (2020)

Components of the LINC and NPC complexes coordinately target and translocate a virus into the nucleus to promote infection
por: Spriggs, Chelsey C., et al.
Publicado: (2022)

Synthetic high-density lipoprotein nanoparticles for the treatment of Niemann–Pick diseases
por: Schultz, Mark L., et al.
Publicado: (2019)

Organ Weights in NPC1 Mutant Mice Partly Normalized by Various Pharmacological Treatment Approaches
por: Antipova, Veronica, et al.
Publicado: (2022)

Inter-domain dynamics drive cholesterol transport by NPC1 and NPC1L1 proteins
por: Saha, Piyali, et al.
Publicado: (2020)

Degradation of lipid droplet-associated proteins by chaperone-mediated autophagy facilitates lipolysis
por: Kaushik, Susmita, et al.
Publicado: (2015)

Preliminary Study of Vibrotactile Feedback during Home-Based Balance and Coordination Training in Individuals with Cerebellar Ataxia
por: Jabri, Safa, et al.
Publicado: (2022)

Going their own way in the NPC
por: Leslie, Mitch
Publicado: (2007)

Simulations of NPC1(NTD):NPC2 Protein Complex Reveal Cholesterol Transfer Pathways
por: Hodošček, Milan, et al.
Publicado: (2018)

Progress Toward Eradication of Polio — Worldwide, January 2011–March 2013
Publicado: (2013)

Progress Toward Polio Eradication — Worldwide, January 2016–March 2018
por: Khan, Farrah, et al.
Publicado: (2018)

Progress Toward Polio Eradication — Worldwide, January 2017–March 2019
por: Greene, Sharon A., et al.
Publicado: (2019)

Progress Toward Polio Eradication — Worldwide, January 2018–March 2020
por: Chard, Anna N., et al.
Publicado: (2020)

Progress Toward Poliomyelitis Eradication — Worldwide, January 2021–March 2023
por: Lee, Scarlett E., et al.
Publicado: (2023)

Species-specific differences in NPC1 protein trafficking govern therapeutic response in Niemann-Pick type C disease
por: Schultz, Mark L., et al.
Publicado: (2022)

The cholesterol transporter NPC1 is essential for epigenetic regulation and maturation of oligodendrocyte lineage cells
por: Kunkel, Thaddeus J., et al.
Publicado: (2023)

Niemann-Pick C1 (NPC1)/NPC1-like1 Chimeras Define Sequences Critical for NPC1’s Function as a Filovirus Entry Receptor
por: Krishnan, Anuja, et al.
Publicado: (2012)

Targeting Ion Channels and Purkinje Neuron Intrinsic Membrane Excitability as a Therapeutic Strategy for Cerebellar Ataxia
por: Huang, Haoran, et al.
Publicado: (2023)

Acat1/Soat1 knockout extends the mutant Npc1 mouse lifespan and ameliorates functional deficiencies in multiple organelles of mutant cells
por: Rogers, Maximillian A., et al.
Publicado: (2022)

Enrichment of NPC1-deficient cells with the lipid LBPA stimulates autophagy, improves lysosomal function, and reduces cholesterol storage
por: Ilnytska, Olga, et al.
Publicado: (2021)

RNA Export through the NPC in Eukaryotes
por: Okamura, Masumi, et al.
Publicado: (2015)

OVOL2 in metastasis prevention in NPC
por: Song, Kyung-A, et al.
Publicado: (2018)

Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 and Ebola virus interactions
por: Zhao, Yuguang, et al.
Publicado: (2016)

Vulnerability of Human Cerebellar Neurons to Degeneration in Ataxia-Causing Channelopathies
por: Bushart, David D., et al.
Publicado: (2022)

Loss of NPC1 enhances phagocytic uptake and impairs lipid trafficking in microglia
por: Colombo, Alessio, et al.
Publicado: (2021)

The effect of changing topography on the coordinated marching of locust nymphs
por: Amichay, Guy, et al.
Publicado: (2016)

ALS/FTD-associated mutation in cyclin F inhibits ER-Golgi trafficking, inducing ER stress, ERAD and Golgi fragmentation
por: Ragagnin, Audrey M. G., et al.
Publicado: (2023)

Immune surveillance of nasopharyngeal carcinoma (NpC)
por: Oluwadara, Oluwadayo, et al.
Publicado: (2011)

Nup82 complex puts the P in NPC
por: Short, Ben
Publicado: (2015)

Hepatic and neuronal phenotype of NPC1(−/−) mice
por: Santiago-Mujica, Estibaliz, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_p4njq5al5ve9qqjeq75p1fj9j0