What is Wolfram Syndrome ?

Dr Benjamin Delprat is a Research Director at the French National Institute of Health, in INSERM Montpellier, France.

He specialized his research with a strong focus on the Wolfram Syndrome.

Deafness is the most common sensory disorder. Indeed 1/700 newborns are deaf.

Wolfram Syndrome is a neurodegenerative disease associating diabetes mellitus and insipidus, optic atrophy and deafness.

Very interestingly, the communication between ER and mitochondria, a micro domain of contact of this two organelles named MAMs (Mitochondria-Associated Membranes), is impaired in Wolfram Syndrome. 

Dr Delprat is using complementary models such as the zebrafish to better understand the structuration and function of these MAMs.

Zebrafish have become a valuable model organism in scientific research, because they share significant genetic and physiological similarities with humans, making them an excellent model to investigate various genetic and developmental disorders.

The rapid reproduction and cost effectiveness of zebrafish make them an ideal organism for lare-scale studies aimed at uncovering novel insights into Wolfram Syndrome pathophysiology and developing targeted treatments.

The final goal of Dr Delprat's research is to identify a treatment that will correct the anomalities of this communication.

Related Publications :


Nature 2018 -    Wolfram syndrome: MAMs’ connection?

2022 - Activation of the sigma-1 receptor chaperone alleviates symptoms of Wolfram syndrome in preclinical models



Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines







Back to the list