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15 May, 2019 - 13:58 By Kate Sweeney

Iceni leads €4m consortium to tackle Glycogen Storage Disease

Iceni Diagnostics

New insights into the structure of sugars, being offered by Iceni Diagnostics in Norwich, could help alleviate the symptoms of Glycogen Storage Disease (GSD) in children; the disease affects all the organs and can be terminal. 

Excess sugar in the body is stored in the form of glycogen in the liver. If the mechanism for controlling storage is impaired then either too much or too little sugar is released into the blood, which can have a massive impact on the body. 

Sugar specialist Iceni Diagnostics has just won funding as part of a €4 million pan-European consortium looking at the reasons why glycogen control is disrupted in the body. 

It aims to develop new diagnostic markers for GSD which would provide early detection of the problem and to identify potential dietary solutions that can alleviate the symptoms.

Professor Rob Field, co-founder of Iceni Diagnostics, says that sugar chemistry is complex but the outcomes of the study could be significant. He says: “Our concern is for the children that have GSD, a rare genetic condition that impacts their ability to store glycogen and it builds up in other organs. 

“This condition has strong parallels with diabetes, which also results from issues with glucose metabolism, so our work could also benefit diabetics.”

Iceni has expertise in determining these structures and has developed a deep knowledge of sugar chemistry from its other work, which is focussed on the development of carbohydrate-based therapeutics and point-of-care diagnostics. 

Prof. Field added: “At the moment not enough is known about the relationship between the gene sequence and the disease state to identify the genetic markers. So we are approaching the problem from a different perspective. We are looking to understand the structure of glycogen and the proteins that act on it, in order to assess the impact of the genetic mutations on both of these classes of biomolecule. 

“There will be opportunities for interventions as it is known that diet can be used to control diabetes. These children have problems metabolising glucose so could potentially benefit from different types of starch that are digested more slowly lower in the gut.” 

Iceni is a spinout from the John Innes Centre, where Prof. Field’s group runs a substantial programme looking at how plants store glucose as starch. This includes work on resistant starches found naturally in crops such as peas, which could be used within a prescriptive diet to control sugar levels. 

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