Gangliosidosis refers to a group of inherited metabolic diseases that are caused by deficiency of different proteins that are needed to break fatty substances down referred to as lipids. It is characterised by an excess of build-up of lipid materials to harmful levels in the central as well as peripheral nervous system, especially in the nerve cells. These genetically different disorders tend to develop when both the parents pass the same mutated gene that regulates the protein to the offspring.
Those children who are suffering from early infantile GM1 tend to often die by the age of 3 years from pneumonia or cardiac complications. Those children who have an early onset form of Tay-Sachs disease tend to often die by the time they are 4 years old because of recurring infections. Those children who are suffering from Sandhoff disease tend to generally die by the time they are 3 years old because of respiratory infections.
Read more articles on Gangliosidoses.