Patients with ML III c retain the most phosphorylation activity of the three disorders and have the mildest course. In fibroblasts and other mesenchymal cells of the patients, lysosomal acid hydrolases which lack the Man-6-P recognition marker are not properly sorted to the lysosome but rather secreted into the patient��s blood stream. The resultant low intracellular levels of these acid hydrolases lead to lysosomal dysfunction and accumulation of Rotigotine undegraded material. However, it has been shown that some non-mesenchymal cells have the ability to traffic lysosomal hydrolases to lysosomes via Man-6-Pindependent mechanisms. Thus, the level of some lysosomal hydrolases has been reported to be normal or near normal in total brain, liver and muscle of ML II patients. In spite of this, these patients exhibit substantial neurologic manifestations including early cessation of neuromotor development, limited verbal expression, and moderate intellectual disability. Liver fibrosis and its sequelea of cirrhosis and hepatocellular carcinoma are responsible for 29,000 deaths a year in the United States, making it the 12th leading cause of death. Injury to the liver results in a wave of cytokine mobilization, many of which are secreted by Kupffer cells, the liver��s resident macrophages. These cytokines and transforming growth factor-b1 ) then activate HSCs, which deposit extracellular matrix as part of the wound repair response. With chronic injury, ongoing inflammation and HSC activation results in accumulation of scar tissue and eventual decreased liver function. In the Bupivacaine hydrochloride uninjured liver, quiescent HSCs behave like pericytes, surrounding the endothelium of the sinusoids. However, upon injury-induced activation, HSCs become the primary collagenproducing cell in the fibrotic liver. HSC activation, in response to platelet derived growth factor BB and TGFb, is well characterized; however, G-protein coupled receptor signaling pathways also influence their behavior during fibrosis. AngII, ET-1, and norepinephrine have been implicated in promoting HSC activation and thus fibrosis. Therefore, modulating signaling downstream of GPCRs may represent a novel therapeutic target in liver fibrosis, potentially preventing HCC.