Support our Foundation of Myology project
Platforms and Technology Units
Cell immortalisation
Since it was set up in 2007, the MyoLine human cell immortalisation platform has generated more than 200 human myoblast lines isolated from patients with more than 36 different diseases (DMD, LGMD, FSHD, SMA…)
MyoLine platform
The MyoLine platform is headed by Anne Bigot, a researcher at the Institute’s Center of Research in Myology. For several years, MyoLine has been making cell lines available to the scientific community on a collaborative basis, and many international laboratories are already using them.
With the development of many innovative therapeutic approaches for genetic diseases, targeting DNAmacromolécule complexe, l’ADN est le support de l’hérédité (gènes). C’est le constituant des chromosomes. L’ADN est organisé en double hélice (deux brins complémentaires) et constitué de nucléotides de quatre types : adénine, guanine, cytosine et thymine., RNAmacromolécule constituée d’une seule chaîne de nucléotides (simple brin) résultant de la transcription (copie) de l’ADN. or proteins, muscle cells isolated from patients, known as myoblasts, are an ideal in vitro« Dans le verre », expériences réalisées en laboratoire en dehors de l’organisme. model for evaluating these approaches in neuromuscular diseases. These cells have several advantages: they are easy to work with, they reduce the number of animal experiments, and they carry the patient’s exact mutationModification soudaine et transmissible du matériel génétique. Elle peut être spontanée ou induite par des agents dits » mutagènes » (radiations, produits toxiques,…). in their own genetic background.
These in vitro approaches do have limits, however: human somatic cells have a limited proliferation capacity, governed by the mitotic clock, and reach replicative senescence after a set number of divisions. This proliferation limit is reached even earlier in degenerative diseases. The MyoLine platform neutralises the replicative senescence of human muscle cells using a double transduction with lentiviral vectors, thereby creating immortal human cell lines.
More than 200 human myoblast lines
Since it was set up in 2007, the MyoLine human cell immortalisation platform has generated more than 200 human myoblast lines isolated from patients with more than 36 different neuromuscular diseases (DMD, LGMD, FSHD, SMA…). As access to muscle biopsies can be limited for some diseases, it has also developed the immortalisation of skin fibroblasts. These immortalised fibroblasts are then transduced with an inducible MyoD transcriptionTransfert de l’information génétique d’un gène à partir d’une molécule d’ADN en ARN messager (transcrit). factor, and these myoconverted cells form myotubes and express muscle markers in the same way as muscle cells.
The loss of muscle mass in patients with neuromuscular diseases is often accompanied by its replacement with fibrous and/or fatty tissue. In recent years, research in this field has focused on the role played by fibro-adipogenic progenitors (FAPs), because of their ability to differentiate into adipocytes or fibroblastic cells.
To support this research, MyoLine is currently developing FAP models isolated from a range of muscular dystrophies.
Where the consent signed by the donor allows it, the cell lines can also be used under material transfer agreements for the development of therapeutic tools by private partners.
Team members
Anne Bigot, research project manager, platform manager
Mona Bensalah, research engineer (IR)
Kamel Mamchaoui, research engineer (IR)
Vincent Mouly, emeritus research director
Jessica Ohana, study engineer (IE)