Zymenex hosts the EU sponsored Alpha-mannosidosis Hue-Man consortium meeting, which takes place in Denmark during the days of May 9 – 10, 2008. The Hue-Man project group consists of leading scientists and clinicians from prominent universities and hospitals from around Europe, who together with Zymenex, have in 2006, received a 3-year 6th framework EU grant of € 3.2 million. The group is devoting their efforts to investigate and establish clinical parameters in the Alpha-mannosidosis mouse model and to perform a natural history study of the human disease in patients, in order to define clinical endpoints for the upcoming first clinical trials in man.
The Hue-Man project takes and expands the knowledge obtained by the joint research group from the three year 5th framework EU grant supported EURAMAN project, which successfully established an enzyme replacement therapy for a mouse model of Alpha-mannosidosis and demonstrated correction of storage in many tissues including brain, after administration of the lysosomal acid a-mannosidase enzyme (rhLAMAN) Lamazym, developed by Zymenex.
About the disease
The disease is due to a deficiency of the a-mannosidase enzyme and affects approximately 500 patients worldwide. Alpha-mannosidosis is a rare inborn disorder that results in mental retardation, skeletal changes, hearing loss, recurrent infections and progression to early death. The children are often born apparently normal and their conditions worsen progressively, without any possibility to prevent this evolution. In the children that are born healthy, a therapy initiated at an early age could contribute to a normal development. Today, the most promising therapy for lysosomal storage diseases is enzyme replacement therapy (ERT), here the enzyme lacking in the patient is introduced into the blood stream, from where it is internalized by the cells and reaches the lysosomes, acting as the original missing enzyme.