Premature termination codon readthrough upregulates progranulin expression and improves lysosomal function in preclinical models of GRN deficiency

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Location: Deutsche Nationalbibliothek Frankfurt am Main

ISSN: 1750-1326

Extent: Online-Ressource

Language: Englisch

Notes: online resource.

Bibliographic citation: Premature termination codon readthrough upregulates progranulin expression and improves lysosomal function in preclinical models of GRN deficiency ; volume:15 ; number:1 ; day:16 ; month:3 ; year:2020 ; pages:1-19 ; date:12.2020
Molecular neurodegeneration ; 15, Heft 1 (16.3.2020), 1-19, 12.2020

Creator: Frew, Jonathan
Baradaran-Heravi, Alireza
Balgi, Aruna D.
Wu, Xiujuan
Yan, Tyler D.
Arns, Steve
Shidmoossavee, Fahimeh S.
Tan, Jason
Jaquith, James B.
Jansen-West, Karen R.
Lynn, Francis C.
Gao, Fen-Biao
Petrucelli, Leonard
Feldman, Howard H.
Mackenzie, Ian R.
Roberge, Michel
Nygaard, Haakon B.

Contributor: SpringerLink (Online service)

DOI: 10.1186/s13024-020-00369-5

URN: urn:nbn:de:101:1-2020052918355301555769

Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.

Last update: 14.08.2025, 10:50 AM CEST

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Associated

Frew, Jonathan
Baradaran-Heravi, Alireza
Balgi, Aruna D.
Wu, Xiujuan
Yan, Tyler D.
Arns, Steve
Shidmoossavee, Fahimeh S.
Tan, Jason
Jaquith, James B.
Jansen-West, Karen R.
Lynn, Francis C.
Gao, Fen-Biao
Petrucelli, Leonard
Feldman, Howard H.
Mackenzie, Ian R.
Roberge, Michel
Nygaard, Haakon B.
SpringerLink (Online service)

Other Objects (12)

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

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Premature termination codon readthrough upregulates progranulin expression and improves lysosomal function in preclinical models of GRN deficiency

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Other Objects (12)

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

Lysosomal processing of progranulin

The lysosomal protein cathepsin L is a progranulin protease

Lysosomal dysfunction and microglial hyperactivation in models of progranulin deficiency

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice

The role of nucleotide composition in premature termination codon recognition

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia

Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice

Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

CRISPR-induced exon skipping is dependent on premature termination codon mutations

Correction: Multi-modal proteomic characterization of lysosomal function and proteostasis in progranulin-deficient neurons

Loss of TMEM 106B potentiates lysosomal and FTLD ‐like pathology in progranulin‐deficient mice

Effect of small molecule eRF3 degraders on premature termination codon readthrough

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