Sebia has entered into partnership with Inserm (the French National Institute of Health and Medical Research) and Inserm Transfert, its private subsidiary responsible for marketing the outcomes of its scientific research, to develop and commercialize a unique biological test for measuring free alpha globin chains in beta-thalassaemic patients. The test will indicate the severity index of the disease in patients.

Negotiated by Inserm Transfert, the collaboration calls for Sebia to support the research carried out by Inserm, which will develop the test. In return, Sebia will have the possibility of acquiring exclusive rights to industrially develop and commercialize the test internationally.

The test for quantifying alpha globin chains was developed by unit U779 along with the participation of the research team of Professor Frédéric Galactéros, who heads the red blood cell genetic disease unit at the Henri Mondor Hospital within the Paris Public Hospital Authority. According to Galactéros, the test Sebia and Inserm Transfert will develop will fill a gap in orienting the diagnosis of thalassaemic patients, since existing methods are more laborious, more costly, and are exclusively intended for research laboratories. Thus, it should result in the commercialization of a new test that does not use radioactivity, will provide a better appreciation of patients’ phenotypes, and will be better adapted to the monitoring of treatment. Furthermore, it will be suitable on a routine basis and available to all laboratories.

“This test provides an immediate indication of the severity of the disease, since the results can be available within a day, whereas it took a week with the old methods,” said Kamran Moradkhani, an associate practitioner at Henri Mondor-Chenevier Hospital. “It ensures that diagnosis is oriented towards identifying the different mutations at work and gives us highly relevant indicators for comparing different groups of patients.”

The test is based on the interaction of the free alpha globin chain with the alpha haemoglobin stabilizing protein (AHSP). In the case of beta-thalassaemias, there is a synthesis deficit in the beta chain, which has the effect of reducing the amount of haemoglobin A (HbA) in red blood cells and leading to an imbalance between the numbers of alpha and beta chains. This imbalance leads to a relative excess of alpha chains. These chains are very unstable, and despite the fact that the AHSP plays the role of chaperone (capable of controlling and stabilizing them), in people suffering from beta-thalassaemias, they form precipitates that act like oxidants and damage the cell, resulting in apoptosis (cell death) and inefficient erythropoiesis (cell production).

According to the size of the pool of free alpha chains, one can determine the severity of the disease in thalassaemia patients, but also in patients with a synthesis imbalance in their globin chains.

Source: Sebia