GlaxoSmithKline has linked up with Canada's Angiochem to discover, develop and sell treatments for lysosomal storage disorders, such as Fabry, Gaucher, Pompe and Tay Sachs diseases, Hunter syndrome and other mucopolysaccharidosis.

The firms say that under the collaboration, Angiochem will create new compounds, called EPiC-enzymes, which are "intended to penetrate the blood-brain barrier (BBB) and restore enzyme function in the central nervous system". Current enzyme replacement therapies, notably produced by Sanofi's Genzyme unit and Shire, are unable to restore enzyme function in the CNS "and therefore fail to ameliorate neurological symptoms associated with lysosomal storage diseases (LSDs)", the companies said.

Cashwise, Angiochem is eligible to receive in excess of $300 million, including an upfront fee of $31.5 million, "if GSK accesses the few LSD targets available to the collaboration". The Montreal-headquartered firm is also eligible to receive royalties on future sales of EPiC-enzymes.

Angiochem will initially create an enzyme replacement therapy for a specified lysosomal storage disease while GSK will have the rights to develop and sell the candidate. The agreement allows for expansion of the collaboration to include additional LSD targets.

Angiochem chief executive Jean-Paul Castaigne said the deal "will further validate the wide-ranging potential for our BBB platform across multiple therapeutic areas and classes of compounds". It will also provide his firm with Angiochem "with additional resources to advance our own internal pipeline including other EPiC-enzymes".