It is known that IL1 neurons together with OLQ neurons are responsible for the sensing of a light nose touch and the regulation of spontaneous foraging movements. Therefore, we tested agrin mutants for light nose-touch-avoidance by the eyelash test. Agrin mutants seemed to be as sensitive to touch as the wild type worms, suggesting that absence of agrin does not lead to a complete failure of IL1 function. Furthermore, IL1 neurons did not show any morphological abnormalities in the agr-1; hdEx25 strain. Pain perception is carried out through small diameter nerve fibers to the central nervous system and is also associated with FD. Specifically, it has been demonstrated that FD patients show reduced intraepidermal nerve fiber density and impaired thermal perception. The underlying molecular and functional mechanisms of pain in SFN patients with Fabry disease are still not completely understood. The difficulty in using fresh samples of sensory neuronal human fibers limits disclosure of the FD molecular and functional mechanisms. To overcome this limitation was created a transgenic mouse model for the Isosorbide a-GalA loss of function. Noteworthy, over the recent years, several ion channels, receptors, and regulatory proteins involved in pain signaling transduction pathways at the periphery and central nervous system have been investigated by the generation of both KO and transgenic mice. Specifically, the a-GalA deficient mice Loxistatin Acid appear clinically normal but display an evident accumulation of glycosphingolipids between 3 and 6 months of ag in several organs, similar to that observed in FD. Interestingly, correction of the enzyme deficit and clearance of the accumulated residues occurred in fibroblasts of the knock out mouse model which reflect the high level of analogy on the mechanisms in the pathophysiological process of FD patients. Taken together, these data indicate that a-GalA KO mice are an excellent system model for the study of FD. Previously, Rodrigues and co-workers demonstrated that this knockout mouse has alterations in sensorimotor function and hypoalgesia.