MCD BIO 165A Chapter Notes - Chapter lysosome: Lysosome, Ganglioside, Phagocytosis
22 Nov 2018
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Vid lysosome storage disease: lysosome & physiological turnover of cell constituency, digestion of endocytosis & phagocytosis. Stuff like amino acids & sugars released to cytosol to be used to the cell: 1 substrate can be made up of several different materials; different enzyme tackles a specific materials. If one of enzymes malfunctions -> product not digested -> progressively accumulated. Can have wide range of symptoms affecting wide arrange of organs. E. g. cells with lots of lysosomes, or neuron cells -> cns affected: best studied: tay-sachs disease. Results from deficiency in enzymes responsible for degrading gangliosides, a major component of cell membrane. Has a high incidence among jews of eastern european ancestry. Different substrates have specific enzymes that break them down. Just messing up with 1 enzyme can have severe consequences. Brains affected the most: lots of monocytes in macrophages; liver has specific macrophage called cuffer cell, Spleen also contains monocytes & macrophages storing undigested material -> affects function.
