Author response: Antagonistic roles for Ataxin-2 structured and disordered domains in RNP condensation

Singh, Amanjot; Hulsmeier, Joern; Kandi, Arvind Reddy; Pothapragada, Sai Shruti; Hillebrand, Jens; Petrauskas, Arnas; Agrawal, Khushboo; Rt, Krishnan; Thiagarajan, Devasena; Jayaprakashappa, Deepa; VijayRaghavan, K.; Ramaswami, Mani; Bakthavachalu, Baskar

doi:10.7554/elife.60326.sa2

Cited by 1 publication

(1 citation statement)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, this small reduction, which may indicate a subtle increase in diffuse, less easily measured cytoplasmic labelling, seems unlikely to account for the observed phenotypic differences. As previously shown, the immunoreactivity we observe is specific to the Atx2 protein (Singh et al 2021). Taken together, these observations indicate that as for Drosophila ALS models, progressive cytotoxicity in Drosophila Huntington's models is facilitated by the Atx2-cIDR.…”

Section: Atx2-cidr Is Partially Required For the Progression Of Mhtt-polyq Induced Degenerationsupporting

confidence: 84%

Ataxin-2 Disordered Region Promotes Huntingtin Protein Aggregation And Neurodegeneration In Drosophila Models Of Huntington’s Disease

Huelsmeier

Walker

Bakthavachalu

et al. 2021

Preprint

Self Cite

View full text Add to dashboard Cite

The Ataxin-2 (Atx2) protein contributes to the progression of neurodegenerative phenotypes in animal models of amyotrophic lateral sclerosis (ALS), type 2 spinocerebellar ataxia (SCA-2), Parkinsons Disease (PD) and Huntingtons Disease (HD). However, because the Atx2 protein contains multiple separable activities, deeper understanding requires experiments to address the exact mechanisms by which Atx2 modulates ND progression. Recent work on two ALS models, C9ORF72 and FUS, in Drosophila has shown that a C-terminal intrinsically disordered region (cIDR) of Atx2 protein, required for assembly of RNP granules, is essential for the progression of neurodegenerative phenotypes as well as for accumulation of protein inclusions associated with these ALS models. Here we show that the Atx2-cIDR also similarly contributes to the progression of degenerative phenotypes and accumulation of Huntingtin protein aggregates in Drosophila models of HD. Because Huntingtin is not an established component of RNP granules, these observations support a recently hypothesised, unexpected protein-handling function for RNP granules, which could contribute to the progression of Huntingtons disease and, potentially, other proteinopathies.

show abstract

Section: Atx2-cidr Is Partially Required For the Progression Of Mhtt-polyq Induced Degenerationsupporting

confidence: 84%

Ataxin-2 Disordered Region Promotes Huntingtin Protein Aggregation And Neurodegeneration In Drosophila Models Of Huntington’s Disease

Huelsmeier

Walker

Bakthavachalu

et al. 2021

Preprint

Self Cite

View full text Add to dashboard Cite

show abstract

Author response: Antagonistic roles for Ataxin-2 structured and disordered domains in RNP condensation

Cited by 1 publication

References 0 publications

Ataxin-2 Disordered Region Promotes Huntingtin Protein Aggregation And Neurodegeneration In Drosophila Models Of Huntington’s Disease

Ataxin-2 Disordered Region Promotes Huntingtin Protein Aggregation And Neurodegeneration In Drosophila Models Of Huntington’s Disease

Contact Info

Product

Resources

About