Lysolames

Lysolames

Lysosomes are typically found in animal cells and are responsible for breaking down all kinds of unwanted biomolecules with enzymes and suicide sacs. They are basically the trash men of the cell. Do you think you want a sanitation worker as organelle in chief? Lysosomes are always just fiddling with the trash and only stick with a few other organelles. They are not true team players, it is only about them all the time. We need a true team organelle as chief.

Lysosomes come with many diseases just like the other organelles. A few diseases due to malfunctioning lysosomes would be Tay-Sachs, Canavan, Sandhoff, and GM-1. Tay Sachs is due to a missing enzyme that causes cells to become damaged and results in progressive neurological disorders. There is no cure for this lysosome caused disease yet. It deteriorate the cells very slowly until they die. The brain accumulates a large amount of gangliosides in the brain cell which leads to an early death of those cells. It causes infants to lose the ability to swallow so they are given feeding tubes. This disease makes life much easier for the person suffering from it. Canavan is the deficiency of an enzyme and is one of the most common degenerative cerebral diseases of infants. The disease causes progressive damage to nerve cells in the brain. The children that have it begin to lose motor control, feeding difficulties, abnormal muscle tone, abnormally enlarged head and it can even cause paralysis, blindness, and seizures. Most children do not make it beyond the four year mark, which is very sad that their dysfunctional lysosomes prevent them living a fulfilling life.

A young girl suffering from Tay Sachs Disease

Young boy with Canavan Disease

Sandhoff Disease is a disease that slowly destroys neurons in the brain and spinal cord. It tends to take a few months into infancy to become apparent but when it really does the child slowly loses all motor ability and is no longer able to sit or crawl or even roll over. They also obtain a rather exaggerated response to loud noises. The kids experience seizures, hearing and vision loss, intellectual disability, and even paralysis. Some of these kids can have enlarged organs or abnormal bones and they rarely live much into their childhood. GM-1 has psychomotor retardation and other neurological issues. The beginning of GM-1 has similar traits of Sandhoff but after that stage there is two more. The second stage starts from 18 months to 5 years and they lack the red dot, enlarged organs, or distinct facial features. Their life expectancies shorten too as they may only live into mid childhood now. The third stage is tolerable as it does not show much until teen years and the only issues are involuntary tensing of muscles and an abnormal spinal cord. Their life expectancies tend to vary.

Enlarged organs due to GM-1

Baby suffering from Sandhoff Disease

Hunter Syndrome is a Lysosomal Storage Disease that is caused by a defective enzyme that can lead to many different type of symptoms. Some people with it actually maintain a high standard of life and are actively employed. Others have severe mental impairment and have life expectancies of 15 years due to neurodegeneration or other physical impairments. Due to the storage impairment it can lead to mental retardation and loss of movement in the body. With all these severe diseases brought on by the dysfunctional lysosomes do we really want to have this kind of an organelle in charge? We don't, we want a real leader, someone that doesn't get us killed.

Works Cited:

•  Lewis, Ricki (1997). Human Genetics. Chicago, IL: Wm. C. Brown. p. 247-248. ISBN 0-697-24030-4.
• Chavany C, Jendoubi M. Biology and potential strategies for the treatment of GM2 gangliosidoses. Mol Med Today. 1998 Apr;4(4):158-65. Review. PubMed citation
• Settembre, Carmine; Fraldi, Alessandro; Medina, Dielo L.; Ballabio, Andrea (2013). Signals from the lysosome: a control centre for cellular clearance and energy metabolism. Nature Reviews Molecular Cell Biology 14 (5): 283–296.doi:10.1038/nrm3565. PMID 23609508.
•  Namboodiri, Am; Peethambaran, A; Mathew, R; Sambhu, Pa; Hershfield, J; Moffett, Jr; Madhavarao, Cn (June 2006). Canavan disease and the role of N-acetylaspartate in myelin synthesis. Molecular and cellular endocrinology 252 (1–2): 216–23.doi:10.1016/j.mce.2006.03.016. PMID 16647192.
• NTSAD - Lysosmal Storage Diseases. NTSAD - Lysosmal Storage Diseases. Web. 2 Feb. 2015. http://www.ntsad.org/index.php/lysosmal-storage-diseases.NTSAD - Lysosmal Storage Diseases.NTSAD - Lysosmal Storage Diseases. Web. 2 Feb. 2015. http://www.ntsad.org/index.php/lysosmal-storage-diseases.

Images Cited:

• Pix For Children With Tay Sachs Disease. Pix For Children With Tay Sachs Disease. Web. 2 Feb. 2015. http://pixgood.com/children-with-tay-sachs-disease.html.
• What Is Canavan Disease. Canavan Research Illinois Cure Canavan Disease. Web. 2 Feb. 2015. http://www.canavanresearch.org/whatis.htm
• Landing BH, Silverman FN, Craig GM, Jacoby MD, Lahey ME, Chadwick DL. Familial neurovisceral lipidosis. Am J Dis Child 1964;108:503-22
• Www.sandhoffdisease.co.uk.Www.sandhoffdisease.co.uk. Web. 2 Feb. 2015. http://www.sandhoffdisease.co.uk/.