Model Organisms
This page was produced as an assignment for Genetics 564 an undergraduate capstone course at UW-Madison.
What are model organisms?
Model organisms are non-human species that are widely used to study and understand biological processes. Model organisms share some common characteristics that make them advantageous for use in biological experiments. Model organisms can be easy to maintain and breed in a lab, typically have short generation times, share substantial genetic similarity to humans, or have similar embryonic development to humans [1]. For the study of disease, one of the most important determinants when choosing a model organism is the similarities of disease phenotypes between the organism humans.
Mice and Cockayne Syndrome
There are 4 mice models in the ERCC6 gene causing Cockayne Syndrome. These models all possess components of the Cockayne Syndrome phenotype found in humans. One of the mice models with the allelic composition of ERCC6tm1Gvh / ERCC6tm1Gvh is extremely photosensitive and develops photophobia. The same model coupled with a xeroderma pigmentosum (a disease that is sometimes assocated with CS) mutation of the allelic composition ERCC6tm1Gvh / ERCC6tm1Gvh, XPAtm1Hvs / XPAtm1Hvs shows the premature aging component of the CS phenotype. This same model also shows a complete inactivation of nucleotide excision repair, one of the key processes that the ERCC6 protein plays a role in. [2]
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Analysis of mice viability in proposed CS research
Because of the link between oxidative damage and premature aging, it makes sense to use a model organism that is capable of demonstrating a premature aging phenotype associated with ERCC6 mutations. Base excision repair (BER) is one of the pathways that manage oxidative damage, and using an ERCC6 mutant organism that cannot manage oxidative damage allows for exploration of the role of ERCC6 in oxidative damage repair. As a result, I will propose using mice, because they are an ideal model organism for studying Cockayne Syndrome and oxidative damage.
References
[1] What are model organisms? webpage. http://www.yourgenome.org/facts/what-are-model-organisms
[2] Cockayne Syndrome, MGI Mouse Model Detail. http://www.informatics.jax.org/disease/DOID:2962
Images and Videos
Cover image: http://www.cubocube.com/dashboard.php?a=1179&b=1228&c=103
Mouse: https://www.jax.org/news-and-insights/2008/october/tallyho-jngj-a-polygenic-mouse-model-of-type-2-diabetes
This website was created for Genetics 564 by Zachary Beethem, an undergraduate genetics major at UW-Madison.
He can be reached via email: [email protected]
Date of last website update: April 2017
[1] What are model organisms? webpage. http://www.yourgenome.org/facts/what-are-model-organisms
[2] Cockayne Syndrome, MGI Mouse Model Detail. http://www.informatics.jax.org/disease/DOID:2962
Images and Videos
Cover image: http://www.cubocube.com/dashboard.php?a=1179&b=1228&c=103
Mouse: https://www.jax.org/news-and-insights/2008/october/tallyho-jngj-a-polygenic-mouse-model-of-type-2-diabetes
This website was created for Genetics 564 by Zachary Beethem, an undergraduate genetics major at UW-Madison.
He can be reached via email: [email protected]
Date of last website update: April 2017