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/.

Riboslam

Ribosomes

Ribosomes synthesize proteins in the cell. Pssh, unnecessary. To do this they translate the information in mRNA into peptide chains. They connect amino acid after amino acid. They tend to just hang on to the Rough ER or just float about in the cytoplasm like some lazy bums. I think we can all remember to that fateful night back in that cold winter some years back. Richard Ribosome, my competition, was at a formal party thrown by Governor Cell Wall and the poor guy didn't know his limits and lets just say he ended up spending the night with Chiraq's finest. Man, we never let him forget about this.

Do you really want this man to lead your country?

A mutation in small ribosomal subunits can lead to the disease known as Treacher Collins Syndrome which causes abnormalities in the facial features of the diseased. These individuals can have such abnormal ears and eyes that they can lose their hearing and vision, while it can be life threatening as well as it restricts air pathways. Thanks a lot ribosomes. Ribosomal malfunctions also lead to male infertility and Bowen Conradi Syndrome, just more negatives for this man who thinks he can be a leader. Male infertility is obvious why it is bad, but BCS is pretty severe. It is characterized by both prenatal and postnatal growth retardation, macrocephaly, a distinct facial appearance, extreme psychomotor delay, hip and knee contractures and rockerbottom feet. Those with it usually do not live past two years and if they do they have extreme growth failures. Very severe diseases thanks to ribosomes.

Treacher Collins Syndrome

Distinct Facial Features with BCS

Those were only mutations to small ribosomal subunits but there is disease and dysfunctions with mutations to the larger subunits. Two of these diseases are Alopecia, neurological defects, and endocrinopathy syndrome and Shwachman-Diamond Syndrome. ANE is characterized with hair loss, mental retardation, progressive loss of motor abilities, hypogonadism, short stature and macrocephaly. SDS is characterized with exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal abnormalities and short stature. These two diseases brought on by ribosomal malfunctions is too severe to just tolerate. We cannot have an organelle with this much risk in charge.
 

Shwachman-Diamond Syndrome

Do we really want to have an organelle with this much opportunity to do bad be in charge? Chloroplasts do not harm anyone, we are the ultimate organelle and without them Lawrence and Peter could not have discussed what they would do with a million dollars. Thank you chloroplast, not you ribosomes, you could have hurt Lawrence.

More on the mugshot above. At Cell Wall's party, Ribosome was bragging about some Diamond he had recently created talking about how he was messing with people's bone marrow and skeleton after they purchased his diamond. It was even said that he somehow was causing people to become shorter and making it difficult for them to digest fats. He was mouthing this all off to the wrong guy, he was telling Officer Milton about his scheme and was arrested and sent to Vacuole County for the night. Richard has been put on many black lists due to this arrest and his scheme which is still around today as it is lingering and affecting many people worldwide.

Works Cited:

• Dauwerse JG, Dixon J, Seland S, Ruivenkamp CA, van Haeringen A, Hoefsloot LH, Peters DJ, Boers AC, Daumer-Haas C, Maiwald R, Zweier C, Kerr B, Cobo AM, Toral JF, Hoogeboom AJ, Lohmann DR, Hehr U, Dixon MJ, Breuning MH, Wieczorek D. Mutations in genes encoding subunits of RNA polymerases I and III cause Treacher Collins syndrome. Nat Genet. 2011 Jan;43(1):20-2. doi: 10.1038/ng.724. Epub 2010 Dec 5. PubMed citation
• Benne R, Sloof P (1987). "Evolution of the mitochondrial protein synthetic machinery".BioSystems 21 (1): 51–68. doi:10.1016/0303-2647(87)90006-2. PMID 2446672.
• James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
• Dror Y, Donadieu J, Koglmeier J, Dodge J, Toiviainen-Salo S, Makitie O, Kerr E, Zeidler C, Shimamura A, Shah N, Cipolli M, Kuijpers T, Durie P, Rommens J, Siderius L, Liu JM. Draft consensus guidelines for diagnosis and treatment of Shwachman-Diamond syndrome. Ann N Y Acad Sci. 2011 Dec;1242:40-55. doi: 10.1111/j.1749-6632.2011.06349.x. PubMed citation
• Result Filters.National Center for Biotechnology Information. U.S. National Library of Medicine. Web. 2 Feb. 2015. http://www.ncbi.nlm.nih.gov/pubmed/18439547.

Images Cited:

• Choroby Rzadkie: Zespół Treacher-Collins'a. Choroby Rzadkie: Zespół Treacher-Collins'a. Web. 2 Feb. 2015. http://chorobyrzadkie.blogspot.com/2012/09/zespo-treacher-collinsa.html.
• OMIM Clinical Synopsis - 1170 - ACROFACIAL DYSOSTOSIS SYNDROME OF RODRIGUEZ. OMIM Clinical Synopsis - 1170 - ACROFACIAL DYSOSTOSIS SYNDROME OF RODRIGUEZ. Web. 2 Feb. 2015. http://www.omim.org/clinicalSynopsis/201170
• CDD Congenital Diarreheal Disorders - Prof. Roberto Berni Canani. CDD Congenital Diarreheal Disorders - Prof. Roberto Berni Canani. Web. 2 Feb. 2015. http://www.congenitaldiarrhealdisorders.net/defects_digestion/testo_03_i.aspx.

Centrioles: The Lazy Organelle

The Sleeping Centrioles

Centrioles are cowards. Yes, they may look like churros. Yes, they are involved in the important processes of cell division during prophase, anaphase and telophase which is when they split chromosomes into two and a new nuclear envelope is formed. But apart from that mumbo jumbo they just relax in interphase and do not contribute to the cell anymore and are a threat to human life. They are truly the lazy organelle and only are located in animal cells, plants are important too, I mean if you like oxygen. C'mon lets be honest, they are not the organelle you want to elect to be Chief Organelle. He would just want to sleep in office and only work for a little bit at the beginning of his term. Not the leader this country needs.

They have been linked to many defects as they are essential in the formation of centrosomes and cilia. A defect in the centrosome is common in the formation in tumors and genome instability. Man, when they give a cell poor cilia it can lead to some pretty big issues with that person. Dysfunctional cilia is linked to diabetes, obesity, loss of vision, cystic kidney disease, mental retardation, polydactyly, and short bones.

Cystic Kidney Disease. Thanks a lot centrioles.

Polydactyly, always lending a hand centrioles.

The centrioles have shown to have some importance to the cell but you decide if all these potential risks are worth it? Do you think this organelle should be put in office? Like really, think about it. Ciliary dysfunctions caused by the centrioles are leading to life threatening diseases. The world is already a dangerous place, do we want to really put someone that causes this many issues in charge. According to the CDC, diabetes is the #7 cause of death in America, which happens to be a ciliary dysfunction. 

Ciliary Dysfunction leads to obesity, an increasing problem in America.

A centriole killer.

Works Cited:

•  Eddé, B; Rossier, J; Le Caer, JP; Desbruyères, E; Gros, F; Denoulet, P (1990). Posttranslational glutamylation of alpha-tubulin. Science 247(4938): 83–5. Bibcode:1990Sci...247...83E.doi:10.1126/science.1967194. PMID 1967194.
• S.J. Morrison, A.C. Spradling: Stem cells and niches: mechanisms that promote stem cell maintenance throughout life Cell, 132 (2008), pp. 598–611
• Ciliary biology: understanding the cellular and genetic basis of human ciliopathies. Cardenas-Rodriguez M, Badano JL. Am J Med Genet C Semin Med Genet. 2009 Nov 15; 151C(4):263-80
•  Cui, Cheng ; Chatterjee, Bishwanath ; Francis, Deanne ; Yu, Qing ; SanAgustin, Jovenal T. ; Francis, Richard ; Tansey, Terry ; Henry, Charisse ; Wang, Baolin ; Lemley, Bethan ; Pazour, Gregory J. ; Lo, Cecilia W. (2011).Disruption of Mks1 localization to the mother centriole causes cilia defects and developmental malformations in Meckel-Gruber syndrome. Dis Model Mech 4 (1): 43–56. doi:10.1242/dmm.006262. PMC 3008963.PMID 21045211.Jump up

Images Cited:

• centriole. Art. Britannica Online for Kids. Web. 1 Feb. 2015.  http://kids.britannica.com/comptons/art-5309
•  National Kidney and Urologic DiseasesInformation Clearinghouse (NKUDIC)." Polycystic Kidney Disease. Web. 2 Feb. 2015. .
• English: Conversion of a DICOM-format X-ray from a patient of en:User:Drgnu23, a ten year old male. This is the patient's left hand, posterior-anterior projection. Identifying tags and such have been stripped. The image is his, released under the GFDL. The image was subsequently altered by en:user:Grendelkhan, en:user: Raul654, and en:user:Solipsist., Own Work, http://commons.wikimedia.org/wiki/File:Polydactyly_01_Lhand_AP.jpg
• Wilking, Rick. "Obesity in America. Web. 2 Feb. 2015. http://blogs.reuters.com/photographers-blog/2012/05/22/obesity-in-america/.
• Word of Advice about Diabetes, Especially Juvenile Diabetes. | HowToCureDiabetes."HowToCureDiabetes. Web. 2 Feb. 2015. http://www.howtocurediabetes.org/.