Mapping Motor Neuron Vulnerability in the Neuraxis of Male SOD1G93A Mice Reveals Widespread Loss of Androgen Receptor Occurring Early in Spinal Motor Neurons

Autor: Victoria M. McLeod, Mathew D. F. Chiam, Nirma D. Perera, Chew L. Lau, Wah Chin Boon, Bradley J. Turner

Publikováno v: Frontiers in Endocrinology, Vol 13 (2022)

Sex steroid hormones have been implicated as disease modifiers in the neurodegenerative disorder amyotrophic lateral sclerosis (ALS). Androgens, signalling via the androgen receptor (AR), predominate in males, and have widespread actions in the perip

Externí odkaz: https://doaj.org/article/92fd79cd0a5a4fa5aab2084315b116af

Metabolic Dysfunction in Motor Neuron Disease: Shedding Light through the Lens of Autophagy

Autor: Subhavi De Silva, Bradley J. Turner, Nirma D. Perera

Publikováno v: Metabolites, Vol 12, Iss 7, p 574 (2022)

Amyotrophic lateral sclerosis (ALS) patients show a myriad of energetic abnormalities, such as weight loss, hypermetabolism, and dyslipidaemia. Evidence suggests that these indices correlate with and ultimately affect the duration of survival. This r

Externí odkaz: https://doaj.org/article/6ee2e965f5d54c83b9c8edaaeeae6d12

The Amyotrophic Lateral Sclerosis M114T PFN1 Mutation Deregulates Alternative Autophagy Pathways and Mitochondrial Homeostasis

Autor: Elisa Teyssou, Laura Chartier, Delphine Roussel, Nirma D. Perera, Ivan Nemazanyy, Dominique Langui, Mélanie Albert, Thierry Larmonier, Safaa Saker, François Salachas, Pierre-François Pradat, Vincent Meininger, Philippe Ravassard, Francine Côté, Christian S. Lobsiger, Séverine Boillée, Bradley J. Turner, Danielle Seilhean, Stéphanie Millecamps

Publikováno v: International Journal of Molecular Sciences; Volume 23; Issue 10; Pages: 5694
International Journal of Molecular Sciences
International Journal of Molecular Sciences, 2022, 23 (10), pp.5694. ⟨10.3390/ijms23105694⟩

International audience; Mutations in profilin 1 (PFN1) have been identified in rare familial cases of Amyotrophic Lateral Sclerosis (ALS). PFN1 is involved in multiple pathways that could intervene in ALS pathology. However, the specific pathogenic r

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dcaf345a08f6a94458df416b56509634

Mapping Motor Neuron Vulnerability in the Neuraxis of Male SOD1

Autor: Victoria M, McLeod, Mathew D F, Chiam, Nirma D, Perera, Chew L, Lau, Wah Chin, Boon, Bradley J, Turner

Publikováno v: Frontiers in endocrinology. 13

Sex steroid hormones have been implicated as disease modifiers in the neurodegenerative disorder amyotrophic lateral sclerosis (ALS). Androgens, signalling

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::1a11915f2cbb22d865f702cc03052736
https://pubmed.ncbi.nlm.nih.gov/35273564

Stimulation of mTOR-independent autophagy and mitophagy by rilmenidine exacerbates the phenotype of transgenic TDP-43 mice

Autor: Valeria Rytova, Brittany Cuic, Nayomi Wanniarachchillage, Sophia J Luikinga, Nirma D Perera, Doris Tomas, Bradley J. Turner

Publikováno v: Neurobiology of Disease, Vol 154, Iss, Pp 105359-(2021)

Autophagy, which mediates the delivery of cytoplasmic substrates to the lysosome for degradation, is essential for maintaining proper cell homeostasis in physiology, ageing, and disease. There is increasing evidence that autophagy is defective in neu

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::83e7e9c570ebc5361df7318d7247d51b
https://pubmed.ncbi.nlm.nih.gov/33798740