Why Are Peroxisomal Proteins Important

Cell Bio Bites Topic: Peroxisomes

The Importance of Peroxisomal Proteins in the Human Body

By:
Jeffrey Palumbo
20523300

For
BIOL 130 Tutorial Section 116
Shirley Chan

The Importance of Peroxisomal Proteins in the Human Body
Peroxisomes are small organelles found in almost all eukaryotic cells that contain a variety of enzymes and are responsible for an assortment of essential metabolic processes (Wanders, 2013). Generally, these processes include the β-oxidation of fatty acids and the detoxification of reactive oxygen and nitrogen species. In order for these reactions to occur, housekeeping functions are performed by peroxisomal proteins (Stanley & Williams, 2010). In general, peroxisomal proteins are important to the human body because they play a key role in peroxisome biogenisis and if the proteins misfolds this can lead to serious disorders.
Firstly, peroxisomal proteins are important to the human body because they play a vital role in peroxisome biogenesis.
If proteins do not fold properly, particularly peroxins, there can be serious peroxisomal biogenesis disorders similar to Zellweger Syndrome. Biogenesis disorders can impair brain, liver and kidney formation and functions. This all could be due to mutations in the protein such as amino acid substitutions or deletions (Stanley & Williams, 2010). Another biogenesis disease is primary hyperoxaluria type one. This disorder is caused by the misfolding of alanine-glyoxylate aminotransferase, commonly called AGT. In this case, the mutated AGT still has the PTS1 but almost 90% of it is directed to mitochondria instead of the peroxisome (Erdmann, Schliebs & Wolf, 2010). This results in an increased amount of oxalate which causes chronic kidney disease (Cochat & Rumsby, 2013). The misfolding of peroxisomal proteins can lead to serious diseases and disorders that affect human