Zellweger Syndrome

2011-11-15T00:22:23Z

100136032: Created page with "Zellweger Syndrome is an inherited disease where the lack of proteins in peroxisomes leads to peroxisomal deficiency. This can be caused by a mutation in..."

Zellweger Syndrome is an inherited disease where the lack of proteins in peroxisomes leads to peroxisomal deficiency. This can be caused by a mutation in gene encoding peroxon Pex2 (related to protein import) which will lead to severe abnormalities in major organs (brain, liver & kidneys) and can result in death.<ref>Alberts et al.,2008, Molecular biology of the cell, Fifth ed., Page 723, New York, Garland science</ref>  It is the most severe of the 4 peroxisome biogenesis disorders (PBD) and there is no cure for it. Also. there are only limited treatments available as the metabolic and neurological pathways involved, are affected during fetal development.<ref>http://www.ninds.nih.gov/disorders/zellweger/zellweger.htm</ref>  <references />

Zymogen

2011-01-10T15:36:15Z

100136032: Created page with 'Zymogens are also called proenzymes. They are inactive forms of enzymes used as reycling molecules, produced and secreted, many by the liver, then transported back to …'

Zymogens are also called proenzymes. They are inactive forms of [[Enzyme|enzymes]] used as reycling molecules, produced and secreted, many by the liver, then transported back to the plasma membrane via vesicles ([[Endosome|endosomes]]) and transformed into its active form. <br>The existance of some structral features prevents the enzyme breaking down the cells inwhich they are made. Eg hydrolase, which has extra inhibitory domains at the N-terminus. Once secreted the zymogen is converted to its active form which requires a biochemical change. In some cases this can be [[Hydrolysis|hydrolysis]]. In hydrolase this is achieved as endolysosomes (the fusion of pre existing lysomoes and endosomes), containing active hydrolases which digest the extra inhibitory domains.<ref>Alberts et al.,2008, Molecular biology of the cell, Fifth ed., Page 792/3, New York, Garland science</ref> <br>

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Zellweger Syndrome

Zymogen