Zellweger syndrome is a genetic disorder that is inherited. The disorder is present at birth, and the infant usually succumbs to it within the first year of its birth. The syndrome causes a reduction or complete lack of certain cytoplasmic organelles known as perioxisomes. These organelles help to detoxify the cells and also contain certain important enzymes that are required for the different chemical processes that go on in the cells.

As perioxisomes are more in number in the cells of the liver, kidneys and brain, these organs are the ones that are severely affected. In addition, the disorder also causes very long chain fatty acids and these encapsulate the nerve fibers.

Zellweger syndrome is a rare disorder, and is seen in one live birth out of a hundred thousand live births. It is an autosomal recessive disorder, which basically means that if both the parents have the gene for the syndrome, then there is twenty-five percent chance that their offspring will be born with Zellweger syndrome.

It was in the year 1964 that a doctor named Hans Zellweger described two pairs of siblings who were unrelated having same types of birth defects. Later, two other doctors also found similar cases, and it was them who labeled the disorder as being a cerebro hepato renal syndrome. However, to give credit to Hans Zellweger for his findings, the name of the disorder was changed to Zellweger cerebro hepato renal syndrome, but it is more commonly referred to as Zellweger syndrome.

In the beginning, scientists and doctors assumed that they syndrome was a congenital disorder that caused numerous anomalies in the infant. However, it was only in 1973 that researchers found out that patients suffering from the syndrome did not have perioxisomes in the liver and kidneys. This was first proof that proved that the syndrome was a metabolic disorder, and as such it was re-categorized.

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