Lysosomes are membrane-enclosed organelles that

contain an array of enzymes capable of breaking
down all types of biological polymers—proteins,
nucleic acids, carbohydrates, and lipids. Lysosomes
function as the digestive system of the cell, serving
both to degrade material taken up from outside the
cell and to digest obsolete components of the cell
itself.
When viewed through a microscope lysosomes are
visualized as dense spherical vacuoles, but they can
display considerable variation in size and shape as a
result of differences in the materials that have been
taken up for digestion.
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Primary lysosomes have not started a digestive event
Secondary lysosomes have fused with the membrane
of a phagosome
Tertiary lysosomes are left over particles from
incomplete digestive events. These can be seen in
various organs and are known as “aging pigment”
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All of the lysosomal enzymes are acid hydrolases Acid Hydrolases is an enzyme that speeds up the process of hydrolysis.Lysosome digestion of foreign substance (change slide) .lysosome digestion of self Heterophage. DNA.2) characteristic of the rest of the cytoplasm.Lysosomes contain about 50 different enzymes that can hydrolyze proteins. This is important when discussing the effects of Chloroquine (change slide) This slide shows the two ways which lysosomes function Autophage. and lipids. polysaccharides. RNA. Acid hydrolases are active at an acidic pH of about 5 that is maintained within lysosomes but not at the neutral pH (about 7.

The action of chloroquine on lysosomes accounts for its antimalarial activity.Chloroquine. eventually killing the parasite. spleen. For example. bones. as an agent used in the treatment and prevention of malaria. thereby inactivating many lysosomal enzymes. (change slide) So why else are these lysosomes important? In addition to their function for the body. The lack of the glucocerebrosidase enzyme causes harmful substances to build up in the liver. The substances prevent cells and organs from working properly. Gaucher’s disease (the most common of these disorders) results from a mutation in the gene that encodes a lysosomal enzyme required for the breakdown of glycolipids. . The drug concentrates in the acidic food vacuole of the malaria parasite (Plasmodium falciparum) and interferes with its digestive processes. Cholloquine raises the pH of the lysosomal content. mutations in the genes that encode these enzymes are responsible for more than 30 different human genetic diseases. and bone marrow.

A bone marrow transplant may be needed in severe cases. helps break down long chains of sugar molecules called glycosaminoglycans Without the enzyme. including the heart. This enzyme. inherited disease of metabolism in which a person cannot break down long chains of sugar molecules called glycosaminoglycans Persons with Hurler syndrome do not make a substance called lysosomal alpha-L-iduronidase. Treatment Enzyme replacement therapy for Hurler syndrome adds a working form of the missing enzyme to the body. Symptoms can range from mild to severe. glycosaminoglycans build up and damage organs. The medication. Hurler syndrome is a rare. Hurler syndrome is inherited. called laronidase .Treatment Enzyme replacement therapy is available.

(Aldurazyme). Sanfilippo syndrome Sanfilippo syndrome is an inhertited disorder that makes the body unable to properly break down long chains of sugar molecules called glycosaminoglycans. The treatment has had mixed results. . is given through a vein (intravenously). (just like Hurlers) Sanfilippo syndrome occurs when the substances (enzymes) needed to break down the heparan sulfate sugar chain are missing or are defective. Which type a person has depends on which enzyme is affected. Other treatments depend on the organs that are affected. Bone marrow transplant has been used in several patients with this condition. There are four main types of Sanfilippo syndrome. also called MPS III.  Sanfilippo type A is the most severe form.

There is no specific treatment available for Sanfilippo syndrome. and risk factors Tay-Sachs disease occurs when a protein that helps break down a chemical found in nerve tissue called gangliosides. Symptoms appear most severe in persons with type A Sanfilippo syndrome.  Tay-Sachs disease Tay-Sachs disease is a life-threatening disease of the nervous system passed down through families. IQs may be below 50. . Some patients live longer. Causes. gangliosidesbuild up in cells. Most persons with Sanfilippo syndrome live into their teenage years. Outlook (Prognosis) The syndrome causes significant neurological symptoms. Without this protein. especially nerve cells in the brain. including severe intellectual disability. while others with severe forms die at an earlier age. incidence.

Symptoms usually appear when the child is 3 to 6 months old. Most people with Tay-Sachs have the infantile form.Tay-Sachs disease is caused by a defective gene on chromosome 15. and adult forms. Tay-Sachs has been classified into infantile. depending on the symptoms and when they first appear. . Late-onset Tay-Sachs disease. Eye exam (reveals a cherry-red spot in the macula) Treatment There is no treatment for Tay-Sachs disease itself. In this form. is very rare. the nerve damage usually begins while the baby is still in the womb. which affects adults. only ways to make the patient more comfortable. The disease tends to get worse very quickly. and the child usually dies by age 4 or 5. juvenile.