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19 results on '"Cestra, G"'

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2. G4C2 repeat affects nuclear mRNA export pathway in a cellular model of C9orf72-ALS

4. An inherited TBX3 alteration in a prenatal case of ulnar-mammary syndrome: Clinical assessment and functional characterization in Drosophila melanogaster.

5. Cul-4 inhibition rescues spastin levels and reduces defects in hereditary spastic paraplegia models.

6. C9orf72 Toxic Species Affect ArfGAP-1 Function.

7. Biallelic mutations in RNF220 cause laminopathies featuring leukodystrophy, ataxia and deafness.

8. Chemical chaperones targeted to the endoplasmic reticulum (ER) and lysosome prevented neurodegeneration in a C9orf72 repeat expansion drosophila amyotrophic lateral sclerosis (ALS) model.

9. HDAC1 inhibition ameliorates TDP-43-induced cell death in vitro and in vivo.

10. UsnRNP trafficking is regulated by stress granules and compromised by mutant ALS proteins.

11. TUBB Variants Underlying Different Phenotypes Result in Altered Vesicle Trafficking and Microtubule Dynamics.

12. A Lipophilic 4-Phenylbutyric Acid Derivative That Prevents Aggregation and Retention of Misfolded Proteins.

13. Recessive mutations in the neuronal isoforms of DST, encoding dystonin, lead to abnormal actin cytoskeleton organization and HSAN type VI.

15. Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice.

16. Control of mRNA Translation in ALS Proteinopathy.

17. nArgBP2 regulates excitatory synapse formation by controlling dendritic spine morphology.

19. Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.

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