LAMA2-related muscular dystrophy alters Schwann cell calcium homeostasis and increases peripheral nerve inflammation

Hypomorphic Lama2dy2J/dy2J mice show adult sciatic nerve defects with patchy loss of myelinated axons and abnormal myelin architecture, linked to altered calcium signaling.

• Why it matters: LAMA2-related muscular dystrophy impacts peripheral nerves, but how LAMA2 deficiency affects nerve integrity and molecular pathways beyond early postnatal development is unclear.
• What they did: Across postnatal life, sciatic nerve structure was assessed in Lama2dy2J/dy2J mice, and bulk RNA sequencing plus Fura Red calcium imaging in primary Schwann cell cultures were used to test calcium and immune programs.
• The result: LAMA2-deficient nerves display immune-related transcriptional signatures and increased CD45+CD11b+ myeloid-cell infiltration, with decreased intracellular calcium in Schwann cells while ATP-evoked responses remain preserved.

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