LING 530 Lecture Notes - Lecture 3: Fabry Disease, Christian De Duve, Neuroglia

• School: McGill University
• Department: Linguistics
• Course: LING 530
• Professor: Francisco Martinez

Document Summary

Synopsis: cofactors for lysosomal enzymes, secretion-recapture pathway, plasma membranes and lipid rafts, molecular genetics, microglia, blood-brain barrier (bbb, causes of lysosomal storage, residual enzymatic activity, effects of lysosomal storage. Cofactors for lysosomal enzymes: prosaposin also known as psap* is a protein which in humans is encoded by the psap gene, this highly conserved glycoprotein is a precursor for. 4 cleavage products: saposins a, b, c, d: saposins a-d localize primarily to the lysosomal compartment where they facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. Extra notes: prosaposin enables the metabolism of sphingolipids, prosaposin is a precursor for four products- Extra notes: gm2 activator protein- first one that was discovered and that is well known, among the 20 tay sach"s patients, only 2 were due to activator protein but the clinical picture was exactly the same. Degradation of selected sphingolipids in the lysosomes of the cells. Activator proteins required for the respective degradation step in vivo are indicated.