- BOON. _ Ryan Daniel.
Passed away on Dec. 7
Aged 12 years
Members of the Rotary Club,
Berwick and their families are
deeply saddened by the death of
your precious son and brother
Ryan. Our love and thoughts are
with Eric, Wendy, Jessica,
Brenden and all the family.
The Mucopolysaccharidoses (MPSs) are rare genetic disorders in children and adults. They involve an abnormal storage of mucopolysaccharides, caused by the absence of a specific enzyme. Without the enzyme, the breakdown process o f mucopolysaccharides is incomplete. Partially broken down mucopolysaccharides accumulate in the body's cells causing progressive damage. The storage process can affect appearance, development and the function of various organs of the body. Eac h MPS disease is caused by the deficiency of a specific enzyme.
Lysosomal Storage Disorders
The MPS diseases are part of a larger group of disorders known as Lysosomal Storage Disorders (LSDs). The combined incidence of LSDs in the population is 1 in 5,000 live births. Each of the more than 40 individual LSDs result in a deficiency i n the activity of a specific protein (enzyme) which is normally present in each of the billions of cells that make up our bodies.
The role of lysosomes is to degrade waste material made by these cells into simpler products for re-use. This process of waste removal requires the sequential action of a number of enzymes. If one of these enzymes is present in insufficient amo unts, the recycling process cannot proceed and the undegraded material remains stored within the lysosome and, therefore, the cell. As the amount of stored material increases over time, normal cellular functioning becomes increasingly impaired , leading to the emergence of clinical symptoms.
Other diseases similar to MPS include the Mucolipidoses, Oligosaccharidoses and Glycosphingolipidoses. The Australian MPS Society includes these and other disorders as part of its "related diseases" and aims to extend its support and assistanc e to families affected by these disorders.
Babies may show little sign of the disorder, but as more and more cells become damaged, symptoms start to appear. Although there are similarities between all the MPS Diseases, each of the individual diseases will result in different effects on t he body.
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1991 - 2003 (12 years) Has 54 ancestors but no descendants in this family tree.
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