1. Pathway and Pathophysiology of MPS


Lysosomal enzyme deficiencies result in progressive accumulation of ‘natural’ (macro-)molecules

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Above is a brief overview on the biochemical and genetic nature of mucopolysaccharidosis. It has to do with the lysosomal biochemistry. This is a cell, showing the endoplasmatic reticulum, the Golgi apparatus and the lysosome putting out from the Golgi apparatus, and the lysosome is a very interesting organelle. The lysosome is there in all the cells of your body to digest material from inside a cell. There is then a mitochondrial  which is not functioning properly, which is a autophagocytosis as a process, or biochemical matter which can be on the outside of the cell or inside of the cell. It is basically not like a garbage can but  more like a recycling factory inside  the cells of your body.


If one of the enzymes in the lysosome involved in the breakdown of these molecules does not function properly, this will result in a progressive accumulation of the macro-molecule that should be degraded by that enzyme. The enzyme doesn’t work and you get accumulation of the substrate of the enzyme.

 

 

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