A Cystine-Rich Whey Supplement (Immunocal(®)) Delays Disease Onset and Prevents Spinal Cord Glutathione Depletion in the hSOD1(G93A) Mouse Model of Amyotrophic Lateral Sclerosis

Antioxidants
Erika K RossDaniel A Linseman

Abstract

Depletion of the endogenous antioxidant, glutathione (GSH), underlies progression of the devastating neurodegenerative disease, amyotrophic lateral sclerosis (ALS). Thus, strategies aimed at elevating GSH may yield new therapeutics for ALS. Here, we investigated the effects of a unique non-denatured whey protein supplement, Immunocal(®), in the transgenic Gly position 93 to Ala (G93A) mutant hSOD1 (hSOD1(G93A)) mouse model of ALS. Immunocal(®) is rich in the GSH precursor, cystine, and is therefore capable of bolstering GSH content. Transgenic hSOD1(G93A) mice receiving Immunocal(®) displayed a significant delay in disease onset compared to untreated hSOD1(G93A) controls. Additionally, Immunocal(®) treatment significantly decreased the rate of decline in grip strength and prevented disease-associated reductions in whole blood and spinal cord tissue GSH levels in end-stage hSOD1(G93A) mice. However, Immunocal(®) did not extend survival, likely due to its inability to preserve the mitochondrial GSH pool in spinal cord. Combination treatment with Immunocal(®) and the anti-glutamatergic compound, riluzole, delayed disease onset and extended survival in hSOD1(G93A) mice. These findings demonstrate that sustaining tissue GSH with Imm...Continue Reading

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Citations

Sep 13, 2019·Free Radical Research·Linda GiblinMichael J Davies
Nov 30, 2019·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·UNKNOWN ALSUNTANGLED GROUP
Jun 30, 2019·Journal of Molecular Neuroscience : MN·Ella ZeldichCarmela R Abraham
Sep 30, 2020·Antioxidants·Montserrat MaríAnna Colell
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Methods Mentioned

BETA
transgenic
genotyping
PCA
protein assay

Software Mentioned

Immunocal ®
Immunocal
CoulArray

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