What question did this study set out to answer?

To elucidate the role of arginine-rich domains and C9orf72-derived dipeptide repeats in liquid-liquid phase separation and stress granule dynamics linked to ALS/FTLD pathogenesis.

March 1, 2017Molecular CellOpen Access

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

View Full Paper

Ask AI

Bookmark

Authors

SBSteven BoeynaemsBaylor University EBElke BogaertUniversity College Ghent DKDénes KovácsUniversity of Szeged

Discussion

Member takes

Implication

In vitro and cellular investigation demonstrates that arginine-rich dipeptide repeats drive aberrant phase separation, suggesting a key pathogenic mechanism in C9orf72 ALS/FTLD.

Key Points

To elucidate the role of arginine-rich domains and C9orf72-derived dipeptide repeats in liquid-liquid phase separation and stress granule dynamics linked to ALS/FTLD pathogenesis.
Assessed the phase-separation capacity of arginine-rich domains and C9orf72-derived dipeptide repeat proteins (DPRs) in vitro.
Expressed arginine-rich DPRs in cultured cells to evaluate spontaneous stress granule assembly and dependence on eIF2α phosphorylation and G3BP.
Arginine-rich DPRs derived from C9orf72 hexanucleotide repeat expansions spontaneously underwent liquid-liquid phase separation and induced the condensation of diverse RNA and stress granule metabolism proteins.
Cellular expression of arginine-rich DPRs triggered spontaneous stress granule formation, which strictly depended on both eIF2α phosphorylation and the assembly factor G3BP.

Cite This Study

Boeynaems et al. (2017) studied this question.

synapsesocial.com/papers/6a72016d8031ec7bb1dd31ee https://doi.org/10.1016/j.molcel.2017.02.013

View Full Paper

Ask AI

Bookmark

Implication

In vitro and cellular investigation demonstrates that arginine-rich dipeptide repeats drive aberrant phase separation, suggesting a key pathogenic mechanism in C9orf72 ALS/FTLD.

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

Discussion

Member takes

Implication

Key Points

Cite This Study

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

Discussion

Member takes

Implication

Key Points

Cite This Study

Also Consider

Also Consider