Fig. 8
From: The pathogenic role of c-Kit+ mast cells in the spinal motor neuron-vascular niche in ALS
Schematic hypothesis about the pathogenic role of mast cells in ALS. c-Kit+ MC precursors infiltrate from the blood through altered microvascular elements that surround the degenerating/damaged motor neurons (MTN) and accumulate in the ALS spinal cord. The c-Kit ligand SCF expressed in reactive astrocytes and motor neurons promotes chemoattraction of c-Kit+ precursors that differentiate into MC bearing granular morphology and expressing typical MC markers exerting a potentially toxic effect on the microvasculature, glial cells, and motor neurons. Pharmacological inhibition of c-Kit reduces the MC number and the microvasculature pathology