Degener, F. (2012) Peroxisomal biogenesis disorders: A review about the molecular background, classification and recent developments. Bachelor's Thesis, Biology.
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Abstract
Peroxisomes are organelles found in virtually all eukaryotic organisms, fulfilling a variety of universal and also very species-specific tasks. In humans, peroxisomes accomplish essential functions, such as β-oxidation of very long chain fatty acids, plasmalogen synthesis and bile acid conjugation. The importance of this organelle in human physiology is emphasized by the multitude of peroxisomal disorders. In order to understand the physiological consequences of peroxisomal diseases in general and peroxisomal biogenesis disorders in particular, the most important aspects of peroxisomes are elucidated, concerning peroxisome biogenesis, proliferation and metabolic functions. The value of an effective classification is highlighted, progressively stimulating the identification of novel mutation causing severe peroxisomal dysfunctions. The very recent finding of a Pex11β dysfunction, surprisingly causing a mild Zellweger syndrome instead of a nonviable or neonatal lethal etiopathology as its subsequent mouse model, will be discussed as well as other substantial developments.
| Item Type: | Thesis (Bachelor's Thesis) |
|---|---|
| Degree programme: | Biology |
| Thesis type: | Bachelor's Thesis |
| Language: | English |
| Date Deposited: | 15 Feb 2018 07:49 |
| Last Modified: | 15 Feb 2018 07:49 |
| URI: | https://fse.studenttheses.ub.rug.nl/id/eprint/10293 |
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