References
- Obrador E, Salvador R, López-Blanch R, et al. Oxidative stress, neuroinflammation and mitochondria in the pathophysiology of amyotrophic lateral sclerosis. Antioxidants. 2020;9:901.
- López-Blanch R, Salvador-Palmer R, Estrela JM, Obrador E. An intercellular flow of glutathione regulated by interleukin 6 links astrocytes and the liver in the pathophysiology of amyotrophic lateral sclerosis. Antioxidants. 2021;10:2007.
- Obrador , et al. The link between oxidative stress, redox status, bioenergetics and mitochondria in the pathophysiology of ALS. Int J Mol Sci. 2021.
- de la R, et al. Efficacy and tolerability of EH301 for amyotrophic lateral sclerosis: a randomized, double-blind, placebo-controlled human pilot study. Amyotroph Lateral Scler Frontotemporal Degener. 2019;1--2:115--122.
- Obrador E, Salvador R, Marchio P, et al. Nicotinamide riboside and pterostilbene cooperatively delay motor neuron failure in ALS SOD1G93A mice. Mol Neurobiol. 2021;58:1345–71.
Reference
- Fox G, Fergusson DA, Daham Z, et al. Patient engagement in preclinical laboratory research: a scoping review. EBioMedicine. 2021;70:103484.
References
- Ma XR, Prudencio M, Koike Y, et al. TDP-43 represses cryptic exon inclusion in the FTD-ALS gene UNC13A. Nature 2022;603:124–30.
- Brown A-L, Wilkins OG, Keuss MJ, NYGC ALS Consortium, et al. TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A. Nature 2022;603:131–7.
References
- Bharadwaj S, Ginoya S, Tandon P, et al. Malnutrition: laboratory markers vs. nutritional assessment. Gastroenterol Rep. 2016;4:272–80.
- Ca R, Bapen EM. 2016.
- Chiò A, Moglia C, Canosa A, et al. Cognitive impairment across ALS clinical stages in a population-based cohort. Neurology. 2019;93:e984–e994.
- Ferri A, Coccurello R. 2017;2017:7821672.
- Foster LA, Salajegheh MK. 2019;132:32–7.
- Gandy J. 2019;361.
- Gkiouleka A, Manning A, Smith D, et al. 2019; 9:e022462.
- Hand RK, Albert JM, Sehgal AR. 2019;29:416–27.
- RCP Stroke Guideline. 2016.
- Kurien M, White S, Simpson G, et al. Managing patients with gastrostomy tubes in the community: can a dedicated enteral feed dietetic service reduce hospital readmissions? Eur JClin Nutr. 2012;66:757– 60.
- NICE Guidance 32. 2006 & 42. 2016.
- ProGas. 2015; 14:702–9.
- Shellikeri S, et al. 2017;75:378–92.
- Yedavalli VS, Patil A, Shah P. 2018;8:53.
- Zarei S, Carr K, Reiley L, et al. A comprehensive review of amyotrophic lateral sclerosis. Surg Neurol Int. 2015;6:171.
References
- Bos PH, Lowry ER, Costa J, et al. Development of MAP4 kinase inhibitors as motor neuron-protecting agents. Cell Chem Biol. 2019; 26:1703–15.e37.
- Korobeynikov VA, Lyashchenko AK, Blanco-Redondo B, et al. Antisense oligonucleotide silencing of FUS expression as a therapeutic approach in amyotrophic lateral sclerosis. Nat Med. 2022;28:104–16.
References
- Hammer RP, Tomiyasu U, Scheibel AB. Degeneration of the human Betz cell due to amyotrophic lateral sclerosis. Exp Neurol. 1979;63:336–46.
- Horoupian DS, Thal L, Katzman R, et al. Dementia and motor neuron disease: morphometric, biochemical, and Golgi studies. Ann Neurol. 1984;16:305–13.
- Sephton CF, Tang AA, Kulkarni APNAS. 2014;4:111E4769–78.
- Thammisetty SS, Renaud L, Picher-Martel V, et al. Targeting TDP-43 pathology alleviates cognitive and motor deficits caused by chronic cerebral hypoperfusion. Neurotherapeutics. 2021;18:1095–112.
References
- Dachet L, Ravits S. Predicting disease stage specific spinal motor neurons and glia in sporadic ALS patients. Neurobiol Dis 2019;
- Song C, Wang R, Loeb. Aberrant neuregulin 1 signaling in ALS. J Neuropathol Exp Neurol 2012;
- Song C, Ravits L. Activation of microglial neuregulin1 signaling in the corticospinal tracts of ALS patients with upper motor neuron signs. Amyotroph Lateral Scler Frontotemporal Degener 2014;
- Liu A, Wang S, Loeb S. Slowing disease progression in the SOD1 mouse model of ALS by blocking neuregulin-induced microglial activation. Neurobiol. Dis 2018;
References
- Sephton CF, Good SK, Atkin S, et al. TDP-43 is a developmentally regulated protein essential for early embryonic development. J Biol Chem. 2010;285:6826–34.
- Uchida A, Sasaguri H, Kimura N, et al. Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43. Brain. 2012;135:833–46.
References
- Gabery S, Ahmed RM, Caga J, Kiernan MC, Halliday GM, Petersén Å, et al. Neuropathol. Neuropathol Appl Neurobiol. 2021;47:979–89.
- Etori K, Saito YC, Tsujino N, Sakurai T. Effects of a newly developed potent orexin-2 receptor-selective antagonist, compound 1 m, on sleep/wakefulness states in mice. Front Neurosci. 2014;8:8.
References
- Zangerolamo L, Vettorazzi JF, Rosa LRO, et al. The bile acid TUDCA and neurodegenerative disorders: an overview. Life Sci. 2021;272:119252.
- Khalaf K, Tornese P, Cocco A, Albanese A. Tauroursodeoxycholic acid: a potential therapeutic tool in neurodegenerative diseases. Transl Neurodegener. 2022;11:33.
References
- Lauranzano E, Pozzi S, Pasetto L, et al. Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes. Brain. 2015;138:974–91.
- Pasetto L, Grassano M, Pozzi S, et al. Defective cyclophilin A induces TDP-43 proteinopathy: implications for amyotrophic lateral sclerosis and frontotemporal dementia. Brain. 2021;144:3710–26.
- De Marco G, Lomartire A, Calvo A, et al. Monocytes of patients with amyotrophic lateral sclerosis linked to gene mutations display altered TDP-43 subcellular distribution. Neuropathol Appl Neurobiol. 2017;43:133–53.