Alzheimer
Enzymes
Tay-Sachs disease is a human genetic disorder which results in the accumulation of cells due to the clogging caused by undigested lipids. The organelle involved in this disease is the lysosome.
A disease may develop when one or more of the body's organs malfunction.
In Pompe's disease, the defect lies in the lysosomal enzyme alpha-glucosidase, which leads to the accumulation of glycogen in lysosomes. In Tay-Sachs disease, the defect is in the enzyme hexosaminidase A, which leads to the accumulation of GM2 gangliosides in lysosomes.
The lysosome must be involved in Tay-Sachs disease. Lysosomes are responsible for breaking down cellular waste, including lipids. In Tay-Sachs disease, a deficiency in the enzyme beta-hexosaminidase A leads to the accumulation of lipids in cells, particularly in the lysosomes.
Lysosomes
lysosomes
Lysosomes are the organelles that become defective in these diseases.
Lysosomes are responsible for digesting worn out organelles. If there was not a lysosome, then excess organelles would not be discarded. Tay-Sachs disease is caused by malfunction of lysosomes or one of their digestive proteins.
Addison's disease results from damage to the adrenal glands. This causes under activity of the adrenal cortex with low or no production of the steroid hormones. It is caused by tuberculosis, amyloidosis, severe septicaemia, and damage to the adrenal glands during abdominal surgery. Long term steroid supplements are need to manage this condition.
A disease is when you have a body malfunction or an organ is not working right.
It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J.C.Pompe.The build-up of glycogen causes progressive muscle weakness throughout the body.