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SOD1 Protein Content in Human Central Nervous System and Peripheral Tissues.

Laura Leykam1, P Andreas Jonsson1,2, Karin M E Forsberg3

  • 1Department of Medical Biosciences, Clinical Chemistry, Umeå University, Umeå, Sweden.

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Summary

Gene silencing therapy for amyotrophic lateral sclerosis (ALS) targets superoxide dismutase-1 (SOD1). This study quantises SOD1 levels in the CNS, finding them lower than previously reported and consistent across ALS patients and controls.

Keywords:
ALSSOD1SOD1 protein contentamyotrophic lateral sclerosis

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Area of Science:

  • Neuroscience
  • Biochemistry
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease.
  • Gene silencing therapies aim to reduce toxic superoxide dismutase-1 (SOD1) in the central nervous system (CNS).
  • Normal SOD1 protein levels in the human CNS are debated.

Purpose of the Study:

  • To quantify SOD1 protein content, total protein, and enzymatic activity in CNS and peripheral tissues.
  • To compare SOD1 levels between sporadic/familial ALS patients and controls.
  • To investigate SOD1 levels in CNS areas vulnerable to ALS.

Main Methods:

  • Analysis of SOD1 protein content, total protein, and SOD1 enzymatic activity.
  • Examination of six CNS areas and four peripheral tissues.
  • Comparison between ALS patients (sporadic and familial) and non-ALS controls.

Main Results:

  • Human CNS SOD1 levels are approximately 100 μg/g wet weight (0.16% of total protein), ~10 times lower than previously reported.
  • SOD1 levels in kidney and erythrocytes were similar to CNS levels; liver had higher levels, skeletal muscle lower.
  • No significant differences in SOD1 protein content were observed between ALS patients and controls, or in CNS areas vulnerable to ALS.

Conclusions:

  • SOD1 is an abundant protein in the human CNS, but its levels are lower than previously estimated.
  • SOD1 levels do not differ between ALS patients and controls, suggesting other factors contribute to motor neuron vulnerability.
  • Insufficient control over SOD1 structure and aggregation may be key factors in SOD1 proteotoxicity in vulnerable motor areas.