ACTH (Acthar Gel) Reduces Toxic SOD1 Protein Linked to Amyotrophic Lateral Sclerosis in Transgenic Mice: A Novel Observation

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a complex etiology and pathology that makes the development of new therapies difficult. ACTH has neurotrophic and myotrophic effects, but has not been tested in an ALS mouse model. The G93A-SOD1 mouse model of ALS was used to te...

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Bibliographic Details
Published in:PloS one 2015-05, Vol.10 (5), p.e0125638
Main Authors: Arrat, Hasan, Lukas, Thomas J, Siddique, Teepu
Format: Article
Language:English
Subjects:
Adrenocorticotropic hormone
Adrenocorticotropic Hormone - pharmacology
Adrenocorticotropic Hormone - therapeutic use
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - metabolism
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - prevention & control
Analysis
Animal tissues
Animals
Body Weight - drug effects
Cell Line
Central nervous system
Corticotropin
Disease Models, Animal
Enzyme-Linked Immunosorbent Assay
Etiology
Female
Fibroblasts
Fibroblasts - cytology
Fibroblasts - drug effects
Fibroblasts - metabolism
Genetic engineering
Humans
Kinases
Male
Mice
Mice, Transgenic
Motor Activity - drug effects
Motor Neurons - metabolism
Mutation
Nervous system diseases
Paralysis
Pathology
Rodents
Spinal cord
Spinal Cord - metabolism
Superoxide dismutase
Superoxide Dismutase - analysis
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Superoxide Dismutase-1
Survival Rate
Transgenic mice
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Description
Summary:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a complex etiology and pathology that makes the development of new therapies difficult. ACTH has neurotrophic and myotrophic effects, but has not been tested in an ALS mouse model. The G93A-SOD1 mouse model of ALS was used to test the ability of this drug to delay ALS-like symptoms. We showed that within a specific dose range, ACTH significantly postponed the disease onset and paralysis in the mouse model. To our surprise and of greater significance is that ACTH significantly reduced the levels of soluble SOD1 in the spinal cord and CNS tissues of G93A-SOD1 treated mice as well as cultured fibroblasts.
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0125638