Peroxisomes and Related Diseases
The human body is made up of trillions of microscopic cells, all of which perform specific functions to maintain life. Blood cells transport nutrients throughout the body and fight viruses and infections. Kidney cells remove waste from the body so that it can be excreted through the effort of the cells in the bladder. The list could go on and on.
Even though each cell has a specific function in the human body, every kind of cell has virtually the same structure consisting of a cytoplasm enclosed by a membrane within which are the nucleus and various organelles. The nucleus contains the cell's DNA and the organelles work within the cell to perform its specific function. One important organelle is the peroxisome, which is found in nearly every complex or eukaryotic cell.
What are Peroxisomes?
Peroxisomes are organelles that help the cell metabolize certain chemicals and deal with the waste the cell produces. They are called peroxisomes because they work to convert certain substances into peroxide and then to convert peroxide into water. They are very important to cell function because while it is needed to metabolize certain chemicals and keep the cell functioning, peroxide is also toxic and there must be a way to rid it from the body. Without peroxisomes there could be no way to produce the peroxide necessary for metabolism, nor could there be any way to get rid of it from the body.
- Biology Encyclopedia: Peroxisomes — some basic information on the cell structures called peroxisomes
- Cells Alive — interactive model of the human cell with information on peroxisomes
- Peroxisomes and Nuclei — some basic information on cell organelles including peroxisomes and nuclei
Normal Function of Peroxisomes
For such a small organelle, peroxisomes really perform some complex functions. First, there are enzymes in each peroxisome that take oxygen and then use it to remove hydrogen from certain molecules that have entered the cell. In the process, hydrogen peroxide is produced. Other enzymes in the peroxisome then use the hydrogen peroxide for oxidizing other molecules like alcohol, phenols, and more. This reaction ends up converting the hydrogen peroxide into water, which is not toxic. The kidneys and livers make extensive use of these processes to eliminate waste from the blood stream. Peroxisomes also work to break fatty acids down into their constituent carbon atoms, and they are important for producing certain proteins in the body as well.
- Molecular Expressions: Peroxisomes — information on peroxisomes from the Florida State University
- Peroxisomes and Cell Function — complete overview of peroxisomes and their functions in living cells from the NIH
- Peroxisome Database — extensive information on all things related to peroxisomes
Diseases Related to Peroxisomes
Peroxisome disorders are linked to several diseases in the human body, many of which appear during childhood and are fatal. Adrenoleukodystrophy or Schilder's disease is one of these disorders. Persons who have this disease lack a protein that helps transport fatty acids into the peroxisomes. These fatty acids cannot break down and build up in the body, eventually damaging the brain and nervous system.
A second well-known peroxisomal disease is called Zellweger Syndrome. Persons with this syndrome lack peroxisomes entirely or have a greatly reduced number of them. Fatty acids cannot be broken down on account of this, leading to brain damage and death. There is no cure for Zellweger, and those who have it do not usually live past six months of age.
- Merck: Peroxisomal Disorders — page on peroxisomal diseases from the famous drug company
- Peroxisomal Disorders — a page all about the disorders and diseases related to peroxisomes
- Ians Story: Peroxisomal Disorders — parents of a child with a peroxisomal disease developed this page to educate people about said diseases
- Zellweger Syndrome — information on one of the more well known peroxisomal diseases
Peroxisomes may be small, but they are very important. Having a better understanding of them helps us appreciate the complexity of the human body and the problems that arise when they do not function properly.
