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RGMa collapses the neuronal actin barrier against disease-implicated protein and exacerbates ALS.

Mikito Shimizu1, Naoyuki Shiraishi1, Satoru Tada1,2

  • 1Department of Neurology, Neuroscience, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.

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Repulsive guidance molecule A (RGMa) is elevated in amyotrophic lateral sclerosis (ALS). Targeting RGMa with an antibody reduced disease symptoms and protein buildup in motor neurons by reinforcing the actin barrier.

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Pathology

Background:

  • Repulsive guidance molecule A (RGMa) is known for its role in neuronal development.
  • RGMa's involvement in neurodegenerative diseases like amyotrophic lateral sclerosis (ALS) is not well understood.
  • Neogenin1 is a known mediator of RGMa functions.

Purpose of the Study:

  • To investigate the role of RGMa in the pathogenesis of ALS.
  • To determine if targeting RGMa can ameliorate ALS symptoms.

Main Methods:

  • Measured RGMa levels in cerebrospinal fluid of ALS patients and mSOD1 mice.
  • Administered anti-RGMa monoclonal antibody to mSOD1 mice.
  • Performed histochemical analysis to assess mutant SOD1 protein accumulation.
  • Conducted in vitro studies to evaluate the antibody's effect on cellular uptake.

Main Results:

  • RGMa levels were elevated in both ALS patients and mSOD1 mice.
  • Anti-RGMa antibody treatment improved clinical symptoms in mSOD1 mice.
  • The antibody reduced mutant SOD1 protein aggregation in motor neurons by inhibiting actin depolymerization.
  • In vitro, the antibody prevented mutant SOD1 cellular uptake by strengthening the neuronal actin barrier.

Conclusions:

  • RGMa exacerbates ALS pathology by causing neuronal actin barrier collapse and promoting aberrant protein deposition.
  • Targeting RGMa with antibodies represents a potential therapeutic strategy for ALS.