Team MyoProteos (K.HNIA)

MYOPROTEOS

MyoProteos: Organelle Proteostasis in muscle cell fate and diseases

Myoproteos research program aims to decrypt functioning of proteosatsis network at the organelle level in the context of muscle cell differenciation and maintenance. We built our basic research program to address molecular and pathophysiological mechanisms of muscle atrophy in health and diseases (i.e., congenital myopathies/centronuclear myopathy) and sarcopenia (age-related muscle atrophy ). We explore anabolic cascades (PI3k, mTORC1…) and catabolic processes (ubiquitin proteasome system, autophagy) as well as epigenetic marks to identify defective proteostasis signals during muscle cell fate and maintenance. Our investigations open a route to identify and test novel therapeutic targets in congential myopathies and to decelerate muscle atrophy/dysfunction in sarcopenia/premature aging.

Team Members

Principal scientist

Karim HNIA

INSERM researcher

He develops research programs on how PI-metabolism regulate proteostasis in skeletal muscle and implications in muscle diseases such as myopathies and sarcopenia.

Career path
Master and PhD degrees in biochemistry and molecular biology at university of Montpellier (2002-2006), Postdoc position IGBMC, Strasbourg (2007-2011), junior researcher at the IGBMC (2012) and senior researcher at the I2MC (2016-current)..

Main domains of expertise
Phosphoinositides, proteostasis, trafficking, muscle disease and physiology.

Email:karim.hnia@inserm.fr

Tél/Phone:05 31 22 41 42

Twitter:@hnia_karim

Chercheure

Marie VANDROMME

INSERM researcher

Her current research focuses on the contribution of epigenetic mechanisms to the etiology of X-linked myotubular myopathy, a fatal pediatric disease due to mutations in the gene encoding the phosphoinositide 3-phosphatase MTM1.

Career path

Master’s degree and PhD in Biology&Health at University of Montpellier, France. HDR (University Paul Sabatier. 1995-1997 post-doctoral period at the National Institute for Medical Research, London (England). 1995-current INSERM researcher at the CRBM and IGH, Montpellier (1995-1999), at ENS Lyon (2000-2003), LBCMCP and CBI, Toulouse (2004-august 2021), and I2MC (September 2021-current).

Main domains of expertise

Proliferation and differentiation, skeletal muscle, Cancer, Myopathy, Chromatin, Histone modifications and gene expression, Transcription, Cell and molecular biology.

Email:marie.vandromme@inserm.fr

Tèl/Phone:05 31 22 41 42

PhD student

Mathilde VAUCOURT

PhD Student

Mathilde Vaucourt studies the involvement of autophagy, more precisely mitophagy, during muscle differentiation using physiological and pathological cell models established by genome editing.

Career path

Research Master in Biology and Health at the Faculty of Science and Technology of Nancy (2022).

Main domains of expertise

Cell biology and biochemistry.

Email:mathilde.vaucourt@inserm.fr

Tèl/Phone:05 31 22 41 39

PhD student

Nesrine HIFDI

PhD Student

Nesrine Hifdi studies the role of the ubiquitin ligase Fbxw7 in myogenesis and the link with phosphoinositide metabolism in the context of muscle differentiation using physiological and pathological cell models established by genome editing.

Career path

Master in Biology-Health (specialization in pathophysiology) at the University of Toulouse in 2021.

Main domains of expertise

Cellular and molecular biology, biochemistry of phosphoinositides

Email:nesrine.hifdi@inserm.fr

Tèl/Phone:05 31 22 41 39

Engineer

Marine MARSEILLAC

Engineer

Marine Marseillac is working on the characterization of new PI3Ks inhibitors in different in vitro cellular models.

Career path

Master’s degree in Biology-Health with a specialization in Physiopathology at Paul Sabatier University, Toulouse.

Main domains of expertise

Cellular biology, Molecular biology, Biochemistry.

Email:marine.marseillac@inserm.fr

Tèl/Phone:05 31 22 41 51

Axe de Recherche 1

Coordinateurs:

K. HNIA & M. Vandromme

How MTM1, the missing protein in X-linked centronuclear myopathy, and its phosphoinositides susbtrates/products impact lysosome and mitochondria proteostasis.

Pathophysiological outcomes

X-linked centronuclear myopathy and sacopenic muscle.

Axe de Recherche 2

Coordinateurs:

M. Vandromme & K. HNIA

How MTM1 function could impact epigenetics/transcriptional programs during muscle cell differenciation and maintenance.

Impact physiopathologique

Altered muscle cell differenciation under physiological stress and X-linked centronuclear myopathy, XLCNM).

Axe de Recherche 3

Coordinateurs:

K. HNIA & M. Vandromme

Identify and test druggable targets to restore organelle proteostasis and/or epigenetic alterations in XLCNM mouse/cell models and to decelerate muscle atrophy and dysfunction in sarcopenic models.

publications

PubMed (K. Hnia): https://pubmed.ncbi.nlm.nih.gov/?term=HNIA%20K&sort=date

PubMed (M. Vandromme): https://pubmed.ncbi.nlm.nih.gov/?term=Vandromme+Marie&sort=date

Articles récents

Mansat M, Kpotor AO, Chicanne G, Picot M, Mazars A, Flores-Flores R, Payrastre B, Hnia K, Viaud J. MTM1-mediated production of phosphatidylinositol 5-phosphate fuels the formation of podosome-like protrusions regulating myoblast fusion. Proc Natl Acad Sci U S A. 2024 Jun 4;121(23): e2217971121. PMID: 38805272. https://pubmed.ncbi.nlm.nih.gov/38805272/

Alkhoury C, Henneman NF, Petrenko V, Shibayama Y, Segaloni A, Gadault A, Nemazanyy I, Le Guillou E, Wolide AD, Antoniadou K, Tong X, Tamaru T, Ozawa T, Girard M, Hnia K, Lutter D, Dibner C, Panasyuk G. Class 3 PI3K coactivates the circadian clock to promote rhythmic de novo purine synthesis. Nat Cell Biol. 2023 Jul;25(7):975-988. PMID: 37414850. https://pubmed.ncbi.nlm.nih.gov/37414850/

Gaillard S, Charasson V, Ribeyre C, Salifou K, Pillaire MJ, Hoffmann JS, Constantinou A, Trouche D, Vandromme M. KDM5A and KDM5B histone-demethylases contribute to HU-induced replication stress response and tolerance. Biol Open. 2021 May 15;10(5):bio057729. PMID: 34184733. https://pubmed.ncbi.nlm.nih.gov/34184733/

Anquetil T, Solinhac R, Jaffre A, Chicanne G, Viaud J, Darcourt J, Orset C, Geuss E, Kleinschnitz C, Vanhaesebroeck B, Vivien D, Hnia K, Larrue V, Payrastre B, Gratacap MP. PI3KC2β inactivation stabilizes VE-cadherin junctions and preserves vascular integrity. EMBO Rep. 2021 Jun 4;22(6):e51299. PMID: 33880878. https://pubmed.ncbi.nlm.nih.gov/34184733/

Gavriilidis C, Laredj L, Solinhac R, Messaddeq N, Viaud J, Laporte J, Sumara I, Hnia K. The MTM1-UBQLN2-HSP complex mediates degradation of misfolded intermediate filaments in skeletal muscle. Nat Cell Biol. 2018 Feb;20(2):198-210. Epub 2018 Jan 22. PMID: 29358706. https://pubmed.ncbi.nlm.nih.gov/29358706/

Revues récentes

Nesrine Hifdi, Mathilde Vaucourt, Karim Hnia, Ganna Panasyuk, Marie Vandromme. Phosphoinositide signaling in the nucleus: impacts on chromatin and transcription regulation. Biology of the Cell, 2025 (In Press).

Shaping Striated Muscles with Ubiquitin Proteasome System in Health and Disease.
Hnia K, Clausen T, Moog-Lutz C. Trends Mol Med. 2019 Sep;25(9):760-774. https://pubmed.ncbi.nlm.nih.gov/31235369/

Plus de publications

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