A study conducted by the Picower Institute for Learning and Memory at MIT revealed that a protein called perlecan is critical for maintaining the structural integrity of neurons. The protein helps create a stable yet flexible extracellular matrix around cells, allowing for supportive growth and function. If perlecan is absent, the axons (long projections of neurons) can disintegrate, resulting in the death of synapses (neuronal connections).
- Perlecan is a protein found in both humans and flies, and it is crucial for the structural integrity of neurons. It contributes to the formation of the extracellular matrix, which offers a supportive environment for cell development and function.
- In the absence of perlecan, axons can break apart during development, leading to the death of synapses. This outcome suggests the protein’s role is particularly significant for the durability of developing axons.
- Mutations reducing perlecan can cause Schwartz-Jampel syndrome, characterized by neuromuscular issues and skeletal abnormalities. The study contributes to the understanding of how neurons are affected in this condition.
- The research team found that perlecan is particularly enriched around the neural lamella, a structure that envelops axon bundles and functions similarly to the cladding around a TV cable, maintaining the structure’s integrity.
- Absence of perlecan results in the weakening of the extracellular matrix around nerve bundles, leading to axon breakages and subsequent synapse losses. This structural vulnerability increases the farther an axon segment is from the brain.