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The Effects of Poly-GA and Poly-PR Dipeptide Repeats on Sleep Patterns in

Overview
Journal MicroPubl Biol
Specialty Biology
Date 2024 Mar 18
PMID 38495583
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Abstract

is the most common familial gene associated with amyotrophic lateral sclerosis (ALS). Dipeptide repeats (DPRs) encoded by an expanded nucleotide repeat sequence in the gene were found in the sleep-related neurons of patients, indicating a role of DPRs in ALS-associated sleep disruptions. Poly-GA or poly-PR DPRs were expressed in male to study their effect on sleep Poly-PR expression caused sleep disruptions while poly-GA expression did not. This study validates the use of as an model system for exploring the roles of DPRs in perturbing the underlying molecular mechanisms in sleep regulation.

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References
1.
Khosravi B, Hartmann H, May S, Mohl C, Ederle H, Michaelsen M . Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD. Hum Mol Genet. 2017; 26(4):790-800. PMC: 5409121. DOI: 10.1093/hmg/ddw432. View

2.
Cirelli C, Bushey D . Sleep and wakefulness in Drosophila melanogaster. Ann N Y Acad Sci. 2008; 1129:323-9. PMC: 2715168. DOI: 10.1196/annals.1417.017. View

3.
Majounie E, Renton A, Mok K, Dopper E, Waite A, Rollinson S . Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol. 2012; 11(4):323-30. PMC: 3322422. DOI: 10.1016/S1474-4422(12)70043-1. View

4.
Arnulf I, Similowski T, Salachas F, GARMA L, Mehiri S, Attali V . Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 2000; 161(3 Pt 1):849-56. DOI: 10.1164/ajrccm.161.3.9805008. View

5.
DeJesus-Hernandez M, Mackenzie I, Boeve B, Boxer A, Baker M, Rutherford N . Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011; 72(2):245-56. PMC: 3202986. DOI: 10.1016/j.neuron.2011.09.011. View