Cargando…

Mice deficient in the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1) display a complex retinal phenotype

Neuronal ceroid lipofuscinosis (NCL) type 1 (CLN1) is a neurodegenerative storage disorder caused by mutations in the gene encoding the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1). CLN1 patients suffer from brain atrophy, mental and motor retardation, seizures, and retinal degeneration...

Descripción completa

Detalles Bibliográficos
Autores principales:	Atiskova, Yevgeniya, Bartsch, Susanne, Danyukova, Tatyana, Becker, Elke, Hagel, Christian, Storch, Stephan, Bartsch, Udo
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6775149/ https://www.ncbi.nlm.nih.gov/pubmed/31578378 http://dx.doi.org/10.1038/s41598-019-50726-8

Ejemplares similares

The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function
por: Sapir, Tamar, et al.
Publicado: (2019)

Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1)
por: Segal-Salto, Michal, et al.
Publicado: (2016)

Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons
por: Lyly, Annina, et al.
Publicado: (2007)

Thioesterase PPT1 balances viral resistance and efficient T cell crosspriming in dendritic cells
por: Ou, Pengju, et al.
Publicado: (2019)

Depalmitoylation by Palmitoyl-Protein Thioesterase 1 in Neuronal Health and Degeneration
por: Koster, Kevin P., et al.
Publicado: (2019)

Experimental Therapeutic Approaches for the Treatment of Retinal Pathology in Neuronal Ceroid Lipofuscinoses
por: Bartsch, Udo, et al.
Publicado: (2022)

Palmitoyl Protein Thioesterase 1 Is Essential for Myogenic Autophagy of C2C12 Skeletal Myoblast
por: Yun, Hyeong Rok, et al.
Publicado: (2020)

The Networks of Genes Encoding Palmitoylated Proteins in Axonal and Synaptic Compartments Are Affected in PPT1 Overexpressing Neuronal-Like Cells
por: Pezzini, Francesco, et al.
Publicado: (2017)

Loss of CLN7 results in depletion of soluble lysosomal proteins and impaired mTOR reactivation
por: Danyukova, Tatyana, et al.
Publicado: (2018)

Identification of PPT1 as a lysosomal core gene with prognostic value in hepatocellular carcinoma
por: Tian, Wei, et al.
Publicado: (2023)

Enzymatic activity of palmitoyl‐protein thioesterase‐1 in serum from schizophrenia significantly associates with schizophrenia diagnosis scales
por: Wu, Yaoyao, et al.
Publicado: (2019)

Identification of substrates of palmitoyl protein thioesterase 1 highlights roles of depalmitoylation in disulfide bond formation and synaptic function
por: Gorenberg, Erica L., et al.
Publicado: (2022)

Tandem Mass Spectrometry Assays of Palmitoyl Protein Thioesterase 1 and Tripeptidyl Peptidase Activity in Dried Blood Spots for the Detection of Neuronal Ceroid Lipofuscinoses in Newborns
por: Barcenas, Mariana, et al.
Publicado: (2014)

Palmitoylated acyl protein thioesterase APT2 deforms membranes to extract substrate acyl chains
por: Abrami, Laurence, et al.
Publicado: (2021)

Complex Structural PPT1 Variant Associated with Non-syndromic Canine Retinal Degeneration
por: Murgiano, Leonardo, et al.
Publicado: (2018)

In a Model of Batten Disease, Palmitoyl Protein Thioesterase-1 Deficiency Is Associated with Brown Adipose Tissue and Thermoregulation Abnormalities
por: Khaibullina, Alfia, et al.
Publicado: (2012)

The Batten Disease Palmitoyl Protein Thioesterase 1 Gene Regulates Neural Specification and Axon Connectivity during Drosophila Embryonic Development
por: Chu-LaGraff, Quynh, et al.
Publicado: (2010)

Palmitoyltransferase DHHC9 and acyl protein thioesterase APT1 modulate renal fibrosis through regulating β-catenin palmitoylation
por: Gu, Mengru, et al.
Publicado: (2023)

Construction of efficient and effective transformation vectors for palmitoyl-acyl carrier protein thioesterase gene silencing in oil palm
por: Bhore, Subhash Janardhan, et al.
Publicado: (2011)

Cell-Based Neuroprotection of Retinal Ganglion Cells in Animal Models of Optic Neuropathies
por: Hu, Yue, et al.
Publicado: (2021)

GNS561, a clinical-stage PPT1 inhibitor, is efficient against hepatocellular carcinoma via modulation of lysosomal functions
por: Brun, Sonia, et al.
Publicado: (2021)

Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
por: Atiskova, Yevgeniya, et al.
Publicado: (2021)

High PPT1 expression predicts poor clinical outcome and PPT1 inhibitor DC661 enhances sorafenib sensitivity in hepatocellular carcinoma
por: Xu, Jianjun, et al.
Publicado: (2022)

Toward Engineering the Mannose 6-Phosphate Elaboration Pathway in Plants for Enzyme Replacement Therapy of Lysosomal Storage Disorders
por: Zeng, Ying, et al.
Publicado: (2019)

Retinal hyperreflective foci in Fabry disease
por: Atiskova, Yevgeniya, et al.
Publicado: (2019)

An over-expression system for characterizing Ppt1 function in Drosophila
por: Korey, Christopher A, et al.
Publicado: (2003)

Quantitative analysis of PPT1 interactome in human neuroblastoma cells
por: Scifo, Enzo, et al.
Publicado: (2015)

Seizures in PPT1 Knock-In Mice Are Associated with Inflammatory Activation of Microglia
por: Zhang, Xusheng, et al.
Publicado: (2022)

Current and Emerging Treatment Strategies for Neuronal Ceroid Lipofuscinoses
por: Kohlschütter, Alfried, et al.
Publicado: (2019)

PPT1 inhibition enhances the antitumor activity of anti–PD-1 antibody in melanoma
por: Sharma, Gaurav, et al.
Publicado: (2020)

PPT1 inhibition enhances the antitumor activity of anti–PD-1 antibody in melanoma
por: Sharma, Gaurav, et al.
Publicado: (2022)

The thioesterase APT1 is a bidirectional-adjustment redox sensor
por: Ji, Tuo, et al.
Publicado: (2023)

GFAP hyperpalmitoylation exacerbates astrogliosis and neurodegenerative pathology in PPT1-deficient mice
por: Yuan, Wei, et al.
Publicado: (2021)

PPT1 regulation of HSP90α depalmitoylation participates in the pathogenesis of hyperandrogenism
por: Xue, Tongmin, et al.
Publicado: (2023)

Intravitreal Co-Administration of GDNF and CNTF Confers Synergistic and Long-Lasting Protection against Injury-Induced Cell Death of Retinal Ganglion Cells in Mice †
por: Dulz, Simon, et al.
Publicado: (2020)

Characterization of the acyl-ACP thioesterases from Koelreuteria paniculata reveals a new type of FatB thioesterase
por: Martins-Noguerol, R., et al.
Publicado: (2020)

PPT-DB: the protein property prediction and testing database
por: Wishart, David S., et al.
Publicado: (2008)

BnaPPT1 is essential for chloroplast development and seed oil accumulation in Brassica napus
por: Tang, Shan, et al.
Publicado: (2022)

Saccharomyces cerevisiae Atf1p is an alcohol acetyltransferase and a thioesterase in vitro
por: Nancolas, Bethany, et al.
Publicado: (2017)

Author Correction: The thioesterase APT1 is a bidirectional-adjustment redox sensor
por: Ji, Tuo, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_hlj67fk60t4qhes6eb63uo5ej4