MEK-SHP2 inhibition prevents tibial pseudarthrosis caused by

Animals Schwann Cells / metabolism Pseudarthrosis / pathology Neurofibromin 1 / metabolism Humans Protein Tyrosine Phosphatase, Non-Receptor Type 11 / metabolism Mice, Knockout Stem Cells / metabolism Tibia / pathology Mice Cell Differentiation / drug effects Male Neurofibromatosis 1 / pathology Fibrosis Female Mitogen-Activated Protein Kinase Kinases / metabolism

Journal

Science translational medicine
ISSN: 1946-6242
Titre abrégé: Sci Transl Med
Pays: United States
ID NLM: 101505086

Informations de publication

Date de publication:
26 Jun 2024
Historique:
medline: 26 6 2024
pubmed: 26 6 2024
entrez: 26 6 2024
Statut: ppublish

Résumé

Congenital pseudarthrosis of the tibia (CPT) is a severe pathology marked by spontaneous bone fractures that fail to heal, leading to fibrous nonunion. Half of patients with CPT are affected by the multisystemic genetic disorder neurofibromatosis type 1 (NF1) caused by mutations in the

Identifiants

DOI: 10.1126/scitranslmed.adj1597 PMID: 38924432
pubmed: 38924432
doi: 10.1126/scitranslmed.adj1597
doi:

Substances chimiques

Neurofibromin 1 0
Protein Tyrosine Phosphatase, Non-Receptor Type 11 EC 3.1.3.48
Mitogen-Activated Protein Kinase Kinases EC 2.7.12.2

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

eadj1597

Auteurs

Simon Perrin (S)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.
ORCID: 0000-0003-3598-2617

Sanela Protic (S)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.

Vincent Bretegnier (V)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.

Ingrid Laurendeau (I)

INSERM UMR S1016, Institut Cochin, Université Paris Cité, 75014 Paris, France.
ORCID: 0009-0003-5437-0015

Oriane Duchamp de Lageneste (OD)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.

Nicolas Panara (N)

INSERM UMR S1016, Institut Cochin, Université Paris Cité, 75014 Paris, France.
ORCID: 0009-0003-4383-5593

Odile Ruckebusch (O)

Université Paris Est Creteil, INSERM, IMRB, Plateforme de Cytométrie en flux, 94000 Creteil, France.
ORCID: 0009-0007-2289-6257

Marine Luka (M)

Paris Cité University, Imagine Institute, Laboratory of Inflammatory Responses and Transcriptomic Networks in Diseases, Atip-Avenir Team, INSERM UMR 1163, 75015 Paris, France.
Labtech Single-Cell@Imagine, Imagine Institute, INSERM UMR 1163, 75015 Paris, France.

Cécile Masson (C)

Bioinformatics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163, 75015 Paris, France.
INSERM US24/CNRS UAR3633, Paris Cité University, 75015 Paris, France.
ORCID: 0000-0001-7870-7821

Théodora Maillard (T)

Service de Médecine Génomique des Maladies de Système et d'Organe, Hôpital Cochin, DMU BioPhyGen, Assistance Publique-Hôpitaux de Paris, AP-HP, Centre-Université Paris Cité, F-75014 Paris, France.
ORCID: 0009-0002-7812-0320

Fanny Coulpier (F)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.

Stéphanie Pannier (S)

Department of Pediatric Orthopedic Surgery and Traumatology, Necker-Enfants Malades Hospital, AP-HP, Paris Cité University, 75015 Paris, France.
ORCID: 0000-0002-5581-4699

Philippe Wicart (P)

Department of Pediatric Orthopedic Surgery and Traumatology, Necker-Enfants Malades Hospital, AP-HP, Paris Cité University, 75015 Paris, France.

Smail Hadj-Rabia (S)

Department of Dermatology, Reference Center for Rare Skin Diseases (MAGEC), Imagine Institute, Necker-Enfants Malades Hospital, AP-HP, Paris Cité University, 75015 Paris, France.

Katarzyna J Radomska (KJ)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.
ORCID: 0000-0001-8243-0669

Mohammed Zarhrate (M)

INSERM US24/CNRS UAR3633, Paris Cité University, 75015 Paris, France.
Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163, 75015 Paris, France.

Mickael Ménager (M)

Paris Cité University, Imagine Institute, Laboratory of Inflammatory Responses and Transcriptomic Networks in Diseases, Atip-Avenir Team, INSERM UMR 1163, 75015 Paris, France.
Labtech Single-Cell@Imagine, Imagine Institute, INSERM UMR 1163, 75015 Paris, France.

Dominique Vidaud (D)

INSERM UMR S1016, Institut Cochin, Université Paris Cité, 75014 Paris, France.
Service de Médecine Génomique des Maladies de Système et d'Organe, Hôpital Cochin, DMU BioPhyGen, Assistance Publique-Hôpitaux de Paris, AP-HP, Centre-Université Paris Cité, F-75014 Paris, France.
ORCID: 0009-0004-4777-1387

Piotr Topilko (P)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.
ORCID: 0000-0001-7381-6770

Béatrice Parfait (B)

INSERM UMR S1016, Institut Cochin, Université Paris Cité, 75014 Paris, France.
Service de Médecine Génomique des Maladies de Système et d'Organe, Hôpital Cochin, DMU BioPhyGen, Assistance Publique-Hôpitaux de Paris, AP-HP, Centre-Université Paris Cité, F-75014 Paris, France.
ORCID: 0000-0002-2964-8666

Céline Colnot (C)

Université Paris Est Creteil, INSERM, IMRB, 94000 Creteil, France.
ORCID: 0000-0001-8423-8718

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Cellules › Névroglie › Cellules de Schwann › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Plaies et blessures › Fractures osseuses › Fractures non consolidées › Pseudarthrose › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines tumorales › Protéines suppresseurs de tumeurs › Neurofibromine-1 › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines et peptides de signalisation intracellulaire › Protein Tyrosine Phosphatases › SH2 Domain-Containing Protein Tyrosine Phosphatases › Protein Tyrosine Phosphatase, Non-Receptor Type 11 › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Souris knockout › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Cellules › Cellules souches › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Appareil locomoteur › Squelette › Os du membre inférieur › Os de la jambe › Tibia › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Phénomènes physiologiques cellulaires › Différenciation cellulaire › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Syndromes néoplasiques héréditaires › Neurofibromatoses › Neurofibromatose de type 1 › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Fibrose › MEK-SHP2 inhibition prevents tibial...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines et peptides de signalisation intracellulaire › Protein-tyrosine kinases › Mitogen-Activated Protein Kinase Kinases › MEK-SHP2 inhibition prevents tibial...