Organelle Fact Sheet- Ribosomes
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Organelle Selected Ribosome
History of the ribosome George E. Palade was the first to discover the ribosomes in 1955. He used an electron microscope to study other organelles when he saw them as densely packed particles. The electron microscope uses a beam of electrons
for high resolution images that can produce detailed images of tissues, organelles, and other very small particles. The biologist found them in the cytoplasm and later discovered that they also “preferably associated” with the endoplasmic reticulum. He and his collogues would later receive the Nobel Prize in 1974 for his work on ribosomes. It is now known that ribosomes developed as early as the time of our last common ancestor. The ribosome has always been involved in protein synthesis, but the structure has evolved for modern day organisms and cells. (Petrov, Gulen, et. all)
Function of the ribosomes Ribosomes are crucial for basic functions in a cell, carrying out many roles for an organism. For one, it is the site of protein synthesis, the process in which proteins are made. DNA replication also needs the involvement of ribosomes where translation take place. In this process the ribosomes read the messenger RNA sequences and turns the genetic code in to a long amino acid chain that folds into proteins that are eventually used by the cell. The ribosomes also allow for transfer RNA to attach to the codon sequences which allows for long chains of the proteins to form. Another function of the ribosomes is acting as a catalyst in peptidyl hydrolysis in which peptide bonds form. This peptide bond formation is a metabolic function of the ribosomes that is also important for the synthesis of proteins. Ribosomes synthesize almost all proteins needed by cells including enzymes, actin, and tubulin in the human body, making them their main purpose as an organelle. Hydrogen ion networks and water provide gradients and electrostatic movement for the peptidyl transfers to occur once translation is complete which is how the ribosomes are able to act as a catalyst in the process. Without ribosomes, organisms would not have essential proteins which is a crucial building block for life. Protein of interest Ribosomes synthesize almost all proteins needed by a cell, one including dyskerin. This protein is responsible for maintaining the structure of telomeres, the region of nucleotide sequences located at the end of a chromosome. The telomeres act similar to the aglet of a shoelace, it protects the ends of chromosomes form sticking together, coming apart, or lose genetic material. Dyskerin has also been found to be essential in gene expression since it is involved in the maintenance of telomeres. Dyskerin is
also involved in translation because it keeps the chromosomes intact, regulating the process and maintaining its efficiency by holding the genes in the chromosome together. Genetic disorder
200
Since ribosomes are so important for the synthesis of proteins which are needed for function and metabolism in organisms, when they are defective,
they can cause serious problems. The group of disorders in humans caused by ribosomal abnormalities is call ribosomopathies. One of these ribosomal
diseases includes Dyskeratosis Congenita, an inherited disorder. The disease takes many forms, most commonly X-linked recessive, as it is also more prevalent in men than in women, but some cases include forms that are autosomal dominant and recessive. The disorder is caused by a germline mutation, meaning a gene change occurred in a reproductive cell which then affected the DNA of every cell. The disorder commonly leads to bone marrow failure and in some cases cancer. In one study, a 22-year-
old male presented symptoms of fever and pain, as well as tissue degeneration in his fingers and toes. His bone marrow biopsy revealed some abnormalities, but he seemed to be only slightly anemic. (Shiferaw, Mukka, & et. all). The disorder is more common in men than women because it is X-linked recessive, meaning men only need to receive one copy of the mutation to show the symptoms of discoloration in pigmentation on the skin and anemia. The disorder occurs when dyskerin is
mutated and does not maintain the structure of telomeres. The short and uncontrolled telomeres cause premature cell death as well as chromosomal instability which reduces stem cell reserve. These effects are what lead to bone marrow failure and anemia, as well as the tissue degeneration seen in the case study report. The disorder is very rare and still being researched today to find a treatment to avoid premature mortality. Figure Reference Fox, G.E. (2010). Origin and Evolution of the Ribosome.
Cold Spring Harbor Perspectives in Biology
, 2(9), https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2926754/
Ramakrishnan, V., 2002. Ribosome structure and the mechanism of translation.
Cell
,
108
(4), pp.557-572. https://www.sciencedirect.com/science/article/pii/S0092867402006190
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5. LYSOSOME
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