Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Genomic Translation for ALS Care (GTAC) - WGS

ABSTRACT: Genomic Translation for ALS Care (GTAC) - WGS

PROVIDER: PRJNA853439 | ENA |

REPOSITORIES: ENA

ACCESS DATA

Json Xml

Similar Datasets

Genomic Translation for ALS Care (GTAC) - WGS

Project description:Genomic Translation for ALS Care (GTAC) - WGS

| PRJNA853440 | ENA

Poly-GR impairs translation elongation and increases ribotoxic stress response in C9ORF72-ALS/FTD

Project description:Hexanucleotide repeat expansion in the C9ORF72 gene is the most frequent inherited cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Poly-GR is one of the most toxic dipeptide repeat (DPR) proteins translated from the RNA repeats. It has been shown to affect protein synthesis, but how this contributes to neurodegeneration is not clear. Here, we found that poly-GR inhibits global translation by perturbing translation elongation. We identified that the transcripts with relatively slow elongation rate tend to be further stalled by poly-GR in iPSC-differentiated neurons. This increases ribosome collision and ZAKα-mediated ribotoxic stress response (RSR), which elevates the phosphorylation of p38 and promotes cell death. Knockdown of ZAKα or pharmacological inhibition of p38 can ameliorate the GR toxicity, and improve the survival of C9ORF72-ALS/FTD patient-derived iPSC-neurons. Our study reveals molecular mechanism of poly-GR mediated toxicity on global translatome, and identifies RSR as a potential therapeutic target for C9ORF72-ALS/FTD.

2024-08-23 | GSE230127 | GEO

ALS

Project description:Oryza sativa Japonica Group ALS, Acetolactate synthase (EC4.6.3.8), Acetohydroxy acid synthase, Herbicide resistance (mutated form of rice ALS, OsmALS (W548L/S627I), is expressed in 12 baseline experiment(s);

| Os02g0510200 | ExpressionAtlas

ALS

| Os02g0510200 | ExpressionAtlas

Glucose Hypometabolism Prompts RAN Translation and Exacerbates C9orf72-related ALS/FTD Phenotypes

Project description:Glucose Hypometabolism Prompts RAN Translation and Exacerbates C9orf72-related ALS/FTD Phenotypes

| PRJNA1082654 | ENA

ALS

Project description:Arabidopsis thaliana ALS, Acetolactate synthase, chloroplastic [Source:UniProtKB/Swiss-Prot;Acc:P17597], is differentially expressed in 71 experiment(s);

| AT3G48560 | ExpressionAtlas

ALS

Project description:Arabidopsis thaliana ALS, Acetolactate synthase, chloroplastic [Source:UniProtKB/Swiss-Prot;Acc:P17597], is expressed in 16 baseline experiment(s);

| AT3G48560 | ExpressionAtlas

ALS exome

Project description:ALS exome

| PRJEB27099 | ENA

ALS Compute

Project description:ALS Compute

| PRJNA926000 | ENA

ALS Compute

Project description:ALS Compute

| PRJNA926001 | ENA