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Zebrafish

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RIBOSOMAL PROTEIN MUTATIONS AND p53 LOSS
The one of the most popularly studied organism for analysis of mutations of proteins is zebrafish (Danio rerio).Ribosomal protein mutations are seen in patients suffering from severe anemia or other cell death associated disorders. These mutations have been extensively identified in model organisms like zebra fish. The zebrafish full length p53 protein is 48% identical to the human p53 protein and is highly expressed in embryos. Hence, a study of these intricate relations is carried out in zebrafish.

Ribosomal protein gene is vastly studied in zebrafish for haplosufficiency. Ribosomal protein mutations are seen in patients suffering from Diamond Blackfan anaemia which shows red cell aplasia and bone marrow failure syndrome. As stated earlier, impairment of RP synthesis or mutations in RP can cause stabilization of p53 due to which apoptosis of cells is induced. (Warner & Mckintosh, 2009). In contrast to this observation in humans, it is seen that in zebrafish, p53 is unable to stabilize in spite of ribosomal haplosufficiency and presence of ionizing radiations. This paved the way for an interesting aspect of relationship between ribosomal mutations and p53 regulation. (Antunes et …show more content…

The prominent mechanisms observed in these relationships are discussed below.
In zebra fish, mutations involved in ribosomal proteins, from both small and large subunits, give rise to a rare type of tumor, known as malignant peripheral nerve sheath tumors (MPNSTs). It is seen that loss of function point mutation of p53 (M214K) also gives the same type of tumor morphology.
Therefore, the involvement of ribosomal protein mutations in tumor formation via a p53 degradative mechanism is a prominent possibility. A study was conducted in this regard to identify the link between p53 and ribosomal

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