Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other parts of the cell for recycling. The lysosomes are formed by the fusion of transport vesicles budded from the trans Golgi network with endosomes, which contain molecules taken up by endocytosis at the plasma membrane. Each lysosome is surrounded by a membrane that maintains an acidic pH within the interior. They contain hydrolytic enzymes or acid hydrolases that break down macromolecules such as nucleic acids, proteins, and polysaccharides. This is the key organelle involved in digestion and waste removal. The lysosomes contain enzymes that digest excess or worn out organelles, food particles, and engulfed viruses or bacteria. Lysosomal storage disease is an inherited metabolic disorder resulting from the defects in lysosomal function. Lysosomes function with other organelles by fusing with vacuoles to deposit the cells wastes.
Gaucher's disease is a common lysosomal storage disorder (LSD).
It causes spleen and liver enlargement, blood problems and bone
issues. The signs and symptoms include:
(1) Reduced hemoglobin causing anemia and fatigue
(2) reduced platelet count resulting in bleeding and bruising
(3) reduced lung function causing shortness of breath
(4) enlargement of liver and spleen which results in abdominal
discomfort
(5) reduced bone density leading to bone pain and fractures.
Prevention
There is currently no known method of prevention for Gaucher's
disease. If a person has a family history of Gaucher disease,
genetic testing may be performed to determine if he/she carries the
mutated gene. Although carriers do not have the disease, they can
pass a copy of their mutated gene onto each of their children.
Treatment:
Gaucher's disease may be treated using enzyme replacement therapy
with glucocerebrosidase enzyme. Bone marrow transplants may be
useful for treating serious cases of Gaucher disease. A drug namely
miglustat may be useful for inhibiting the body's ability to
produce glucocerebrosides which results in limiting the buildup of
glucocerebrosides in bodily tissues.
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