scholarly journals A diet deficient in vitamin D 3 delays disease onset in the female, but not the male, G93A mouse model of amyotrophic lateral sclerosis

2011 ◽  
Vol 25 (S1) ◽  
Author(s):  
Jesse Adam Solomon ◽  
Alexandro Gianforcaro ◽  
Mazen Jamal Hamadeh
Keyword(s):  
Vitamin D ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Onset ◽  
Vitamin D 3 ◽  
Lateral Sclerosis

scholarly journals Dietary vitamin D 3 restriction exacerbates disease pathophysiology in the spinal cord of the G93A mouse model of amyotrophic lateral sclerosis (ALS)

2015 ◽  
Vol 29 (S1) ◽  
Author(s):  
Elnaz Moghimi ◽  
Mahshad Kolahdouzan ◽  
Sanjeef Thampinathan ◽  
Jesse Solomon ◽  
Alexandro Gianforcaro ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Vitamin D ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Dietary Vitamin ◽  
Vitamin D 3 ◽  
Dietary Vitamin D ◽  
Lateral Sclerosis

scholarly journals IL-10 based immunomodulation initiated at birth extends lifespan in a familial mouse model of amyotrophic lateral sclerosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Michael R. Strickland ◽  
Kristen R. Ibanez ◽  
Mariya Yaroshenko ◽  
Carolina Ceballos Diaz ◽  
David R. Borchelt ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Chemokine Receptor ◽  
Inflammatory Cytokine ◽  
Disease Onset ◽  
Interleukin 10 ◽  
Inflammatory Chemokines ◽  
Anti Inflammatory ◽  
Anti Inflammatory Cytokine ◽  
Lateral Sclerosis

AbstractInflammatory signaling is thought to modulate the neurodegenerative cascade in amyotrophic lateral sclerosis (ALS). We have previously shown that expression of Interleukin-10 (IL-10), a classical anti-inflammatory cytokine, extends lifespan in the SOD1-G93A mouse model of familial ALS. Here we test whether co-expression of the decoy chemokine receptor M3, that can scavenge inflammatory chemokines, augments the efficacy of IL-10. We found that recombinant adeno-associated virus (AAV)-mediated expression of IL-10, alone, or in combination with M3, resulted in modest extension of lifespan relative to control SOD1-G93A cohort. Interestingly neither AAV-M3 alone nor AAV-IL-10 + AAV-M3 extend survival beyond that of the AAV-IL-10 alone cohort. Focused transcriptomic analysis revealed induction of innate immunity and phagocytotic pathways in presymptomatic SOD1-G93A mice expressing IL-10 + M3 or IL-10 alone. Further, while IL-10 expression increased microglial burden, the IL-10 + M3 group showed lower microglial burden, suggesting that M3 can successfully lower microgliosis before disease onset. Our data demonstrates that over-expression of an anti-inflammatory cytokine and a decoy chemokine receptor can modulate inflammatory processes in SOD1-G93A mice, modestly delaying the age to paralysis. This suggests that multiple inflammatory pathways can be targeted simultaneously in neurodegenerative disease and supports consideration of adapting these approaches to treatment of ALS and related disorders.

scholarly journals A Cystine-Rich Whey Supplement (Immunocal®) Delays Disease Onset and Prevents Spinal Cord Glutathione Depletion in the hSOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

Antioxidants ◽  
2014 ◽  
Vol 3 (4) ◽  
pp. 843-865 ◽  
Author(s):  
Erika Ross ◽  
Aimee Winter ◽  
Heather Wilkins ◽  
Whitney Sumner ◽  
Nathan Duval ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Onset ◽  
Glutathione Depletion ◽  
Lateral Sclerosis

scholarly journals Therapeutic effects of hirsutella sinensis on the disease onset and progression of amyotrophic lateral sclerosis in SOD1 G93A transgenic mouse model

2019 ◽  
Vol 26 (1) ◽  
pp. 90-100 ◽  
Author(s):  
Hai‐Yan Shang ◽  
Jing‐Jing Zhang ◽  
Zhen‐Fa Fu ◽  
Yu‐Fei Liu ◽  
Song Li ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Disease Onset ◽  
Transgenic Mouse Model ◽  
Therapeutic Effects ◽  
Lateral Sclerosis

scholarly journals Deletion of the BDNF Truncated Receptor TrkB.T1 Delays Disease Onset in a Mouse Model of Amyotrophic Lateral Sclerosis

PLoS ONE ◽  
2012 ◽  
Vol 7 (6) ◽  
pp. e39946 ◽  
Author(s):  
Sudhirkumar U. Yanpallewar ◽  
Colleen A. Barrick ◽  
Hannah Buckley ◽  
Jodi Becker ◽  
Lino Tessarollo
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Onset ◽  
Truncated Receptor ◽  
Lateral Sclerosis

scholarly journals Dissociation of disease onset, progression and sex differences from androgen receptor levels in a mouse model of amyotrophic lateral sclerosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Doris Tomas ◽  
Victoria M. McLeod ◽  
Mathew D. F. Chiam ◽  
Nayomi Wanniarachchillage ◽  
Wah C. Boon ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Sex Differences ◽  
Androgen Receptor ◽  
Mouse Model ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Disease Onset ◽  
Disease Course ◽  
Male Mice ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder caused by loss of motor neurons. ALS incidence is skewed towards males with typically earlier age of onset and limb site of onset. The androgen receptor (AR) is the major mediator of androgen effects in the body and is present extensively throughout the central nervous system, including motor neurons. Mutations in the AR gene lead to selective lower motor neuron degeneration in male spinal bulbar muscular atrophy (SBMA) patients, emphasising the importance of AR in maintaining motor neuron health and survival. To evaluate a potential role of AR in onset and progression of ALS, we generated SOD1G93A mice with either neural AR deletion or global human AR overexpression. Using a Cre-LoxP conditional gene knockout strategy, we report that neural deletion of AR has minimal impact on the disease course in SOD1G93A male mice. This outcome was potentially confounded by the metabolically disrupted Nestin-Cre phenotype, which likely conferred the profound lifespan extension observed in the SOD1G93A double transgenic male mice. In addition, overexpression of human AR produced no benefit to disease onset and progression in SOD1G93A mice. In conclusion, the disease course of SOD1G93A mice is independent of AR expression levels, implicating other mechanisms involved in mediating the sex differences in ALS. Our findings using Nestin-Cre mice, which show an inherent metabolic phenotype, led us to hypothesise that targeting hypermetabolism associated with ALS may be a more potent modulator of disease, than AR in this mouse model.

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