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Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models Nature

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models Nature 2025

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The document discusses a study that identifies the RNA lariat debranching enzyme DBR1 as a significant modifier of TDP-43 toxicity in amyotrophic lateral sclerosis (ALS) models. The research reveals that deletion of DBR1 leads to the accumulation of intronic lariats in the cytoplasm, which sequester TDP-43 and prevent its toxic effects on essential cellular RNAs. This suggests that targeting DBR1 could be a potential therapeutic strategy for ALS, particularly in cases associated with TDP-43 aggregation.

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What is the enzyme in ALS?

The researchers discovered that the NADPH oxidase enzyme, an important component in the generation of destructive reactive oxygen species during inflammation, is active in the spinal cords of ALS patients and also in the mouse model of the disease.

What protein builds up in ALS?

Amyotrophic lateral sclerosis (ALS) is a neurological condition that affects motor neurons the nerve cells that control breathing and muscles. Under a microscope, researchers have noticed that the motor neurons of patients with ALS contain excessive aggregation of a protein called TDP-43.

What is the enzyme ALS?

The acetolactate synthase (ALS) enzyme (also known as acetohydroxy acid or acetohydroxyacid synthase, abbr. AHAS) is a protein found in plants and micro-organisms.

What are the RNA binding proteins in ALS?

A number of paraspeckle-enriched RNA-binding proteins, including SFPQ, FUS, EWSR1, TAF15, TARDBP, SS18L1, and HNRNPA1, are mutated in familial cases of ALS as well as other neurodegenerative diseases [17,24,141,142,143,144].

What is the role of TDP-43 in ALS?

TDP-43 self-aggregation or proteinopathy is a hallmark in ALS pathology and can produce consequences with both gain-of-function and loss-of-function characters. TDP-43 has a putative prion-like domain in its C-terminal glycine-rich region and is aggregation-prone.

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What are the enzymes defect in amyotrophic lateral sclerosis?

Deficiency of adenosine deaminase acting on RNA 2 (ADAR2), an enzyme that specifically catalyzes GluA2 Q/R site-editing, has been reported in considerable proportions of spinal motor neurons of the majority of sporadic ALS patients.

What is the Tardbp mutation in ALS?

The TARDBP gene (encoding TDP-43) is among the most commonly mutated ALS-associated genes after C9ORF72, SOD1, and FUS, with nearly 40 missense mutations identified in patients to date accounting for 3% (fALS) and

Why is CK elevated in ALS?

An obvious explanation of raised CK in ALS is striate muscle atrophy resulting from degeneration of the subserving motor neurons. Another possible explanation is upregulation of this enzyme to provide an energy substrate in a hypercatabolic condition.

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models Nature 2025

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What is the enzyme in ALS?

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What are the enzymes defect in amyotrophic lateral sclerosis?

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