MEK inhibitors - novel targeted therapies of neurofibromatosis associated benign and malignant lesions

MEK inhibitors for the treatment of non-small cell lung cancer

(2021) Jing Han et al.   Journal of Hematology & Oncology

Advancing RAS/RASopathy therapies: An NCI‐sponsored intramural and extramural collaboration for the study of RASopathies

(2020) Andrea M. Gross et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

ERK/MAPK signalling pathway and tumorigenesis (Review)

(2020) Yan‑Jun Guo et al.   Experimental and Therapeutic Medicine

Phase II trial of the MEK 1/2 inhibitor selumetinib (AZD6244, ARRY-142886 Hydrogen Sulfate) in adults with neurofibromatosis type 1 (NF1) and inoperable plexiform neurofibromas (PN).

(2020) Geraldine Helen O'Sullivan Coyne et al.   JOURNAL OF CLINICAL ONCOLOGY

Trametinib-based Treatment of Pediatric CNS Tumors

(2020) Megan R. Paul et al.   JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Selumetinib in Children with Inoperable Plexiform Neurofibromas

(2020) Andrea M. Gross et al.   NEW ENGLAND JOURNAL OF MEDICINE

The Use of MEK Inhibitors in Neurofibromatosis Type 1–Associated Tumors and Management of Toxicities

(2020) Laura J. Klesse et al.   ONCOLOGIST

Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism

(2020) Eric Legius et al.   CHILDS NERVOUS SYSTEM

A molecular basis for neurofibroma-associated skeletal manifestations in NF1

(2020) Yun Ma et al.   GENETICS IN MEDICINE

Trametinib for the treatment of recurrent/progressive pediatric low-grade glioma

(2020) Neevika Manoharan et al.   JOURNAL OF NEURO-ONCOLOGY

Modulation of spine fusion with BMP-2, MEK inhibitor (PD0325901), and zoledronic acid in a murine model of NF1 double inactivation

(2020) Justin D. Bobyn et al.   JOURNAL OF ORTHOPAEDIC SCIENCE

Combined inhibition of SHP2 and MEK is effective in models of NF1-deficient malignant peripheral nerve sheath tumors

(2020) Jiawan Wang et al.   CANCER RESEARCH

miR-4286 is Involved in Connections Between IGF-1 and TGF-β Signaling for the Mesenchymal Transition and Invasion by Glioblastomas

(2020) Kuo-Hao Ho et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Response to trametinib treatment in progressive pediatric low-grade glioma patients

(2020) Florian Selt et al.   JOURNAL OF NEURO-ONCOLOGY

Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis

(2020) Zhenyu Cai et al.   World Journal of Surgical Oncology

Activation of receptor tyrosine kinases mediates acquired resistance to MEK inhibition in malignant peripheral nerve sheath tumors

(2020) Jiawan Wang et al.   CANCER RESEARCH

MEK inhibitor resistance mechanisms and recent developments in combination trials

(2020) E. Kun et al.   CANCER TREATMENT REVIEWS

Preclinical Assessment of MEK1/2 Inhibitors for Neurofibromatosis Type 2-Associated Schwannomas Reveal Differences in Efficacy and Drug Resistance Development

(2019) Marisa A Fuse et al.   NEURO-ONCOLOGY

NF1 Somatic Mutation in Dystrophic Scoliosis

(2019) Rebecca L. Margraf et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Targeting β-catenin overcomes MEK inhibition resistance in colon cancer with KRAS and PIK3CA mutations

(2019) Jai-Hee Moon et al.   BRITISH JOURNAL OF CANCER

YAP/TAZ Inhibition Induces Metabolic and Signaling Rewiring Resulting in Targetable Vulnerabilities in NF2-Deficient Tumor Cells

(2019) Shannon M. White et al.   DEVELOPMENTAL CELL

Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial

(2019) Jason Fangusaro et al.   LANCET ONCOLOGY

Trametinib Induces Neurofibroma Shrinkage and Enables Surgery

(2019) Pia Vaassen et al.   NEUROPEDIATRICS

An update on the CNS manifestations of neurofibromatosis type 2

(2019) Shannon Coy et al.   ACTA NEUROPATHOLOGICA

A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway: TRAM-01

(2019) Sébastien Perreault et al.   BMC CANCER

p53 and NF 1 loss plays distinct but complementary roles in glioma initiation and progression

(2018) Phillippe P. Gonzalez et al.   GLIA

Developmental dosing with a MEK inhibitor (PD0325901) rescues myopathic features of the muscle-specific but not limb-specific Nf1 knockout mouse

(2018) Matthew A. Summers et al.   MOLECULAR GENETICS AND METABOLISM

NFM-06. NF106: PHASE 2 TRIAL OF THE MEK INHIBITOR PD-0325901 IN ADOLESCENTS AND ADULTS WITH NF1-RELATED PLEXIFORM NEUROFIBROMAS: AN NF CLINICAL TRIALS CONSORTIUM STUDY

(2018) Brian Weiss et al.   NEURO-ONCOLOGY

SPRINT: Phase II study of the MEK 1/2 inhibitor selumetinib (AZD6244, ARRY-142886) in children with neurofibromatosis type 1 (NF1) and inoperable plexiform neurofibromas (PN).

(2018) Andrea M. Gross et al.   JOURNAL OF CLINICAL ONCOLOGY

Trametinib for progressive pediatric low-grade gliomas

(2018) Maria Kondyli et al.   JOURNAL OF NEURO-ONCOLOGY

Distinct genomic profile and specific targeted drug responses in adult cerebellar glioblastoma

(2018) Hee Jin Cho et al.   NEURO-ONCOLOGY

Dramatic response to trametinib in a male child with neurofibromatosis type 1 and refractory astrocytoma

(2018) Tristan Knight et al.   PEDIATRIC BLOOD & CANCER

The molecular landscape of glioma in patients with Neurofibromatosis 1

(2018) Fulvio D’Angelo et al.   NATURE MEDICINE

Mutation Profiles in Glioblastoma 3D Oncospheres Modulate Drug Efficacy

(2018) Kelli M. Wilson et al.   SLAS Technology

Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1—a consensus overview

(2017) Markku M. Miettinen et al.   HUMAN PATHOLOGY

Selumetinib for children with plexiform neurofibromas

(2017) Talha Khan Burki   LANCET ONCOLOGY

A phase I trial of the MEK inhibitor selumetinib (AZD6244) in pediatric patients with recurrent or refractory low-grade glioma: a Pediatric Brain Tumor Consortium (PBTC) study

(2017) Anuradha Banerjee et al.   NEURO-ONCOLOGY

Frequent AKT1E17K mutations in skull base meningiomas are associated with mTOR and ERK1/2 activation and reduced time to tumor recurrence

(2017) Ümmügülsüm Yesilöz et al.   NEURO-ONCOLOGY

Non-optic glioma in adults and children with neurofibromatosis 1

(2017) Laura Sellmer et al.   Orphanet Journal of Rare Diseases

MEK inhibitors enhance therapeutic response towards ATRA in NF1 associated malignant peripheral nerve sheath tumors (MPNST) in-vitro

(2017) Susan Fischer-Huchzermeyer et al.   PLoS One

Prolonged disease control with MEK inhibitor in neurofibromatosis type I-associated glioblastoma

(2016) M. Ameratunga et al.   JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS

Therapeutic advances for the tumors associated with neurofibromatosis type 1, type 2, and schwannomatosis

(2016) Jaishri O. Blakeley et al.   NEURO-ONCOLOGY

Activity of Selumetinib in Neurofibromatosis Type 1–Related Plexiform Neurofibromas

(2016) Eva Dombi et al.   NEW ENGLAND JOURNAL OF MEDICINE

Combinatorial therapeutic targeting of BMP2 and MEK-ERK pathways in NF1-associated malignant peripheral nerve sheath tumors

(2016) Sidra Ahsan et al.   Oncotarget

RASopathies: unraveling mechanisms with animal models

(2015) G. A. Jindal et al.   Disease Models & Mechanisms

Evaluation of somatic mutations in tibial pseudarthrosis samples in neurofibromatosis type 1

(2015) David W Sant et al.   JOURNAL OF MEDICAL GENETICS

Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas

(2015) Stéphane Goutagny et al.   JOURNAL OF NEURO-ONCOLOGY

Role of Merlin/NF2 inactivation in tumor biology

(2015) A M Petrilli et al.   ONCOGENE

A Combination of rhBMP-2 (Recombinant Human Bone Morphogenetic Protein-2) and MEK (MAP Kinase/ERK Kinase) Inhibitor PD0325901 Increases Bone Formation in a Murine Model of Neurofibromatosis Type I Pseudarthrosis

(2014) J El-Hoss et al.   JOURNAL OF BONE AND JOINT SURGERY-AMERICAN VOLUME

Combined MEK Inhibition and BMP2 Treatment Promotes Osteoblast Differentiation and Bone Healing inNf1Osx−/−Mice

(2014) Jean de la Croix Ndong et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Neurofibromin Deficiency-Associated Transcriptional Dysregulation Suggests a Novel Therapy for Tibial Pseudoarthrosis in NF1

(2014) Nandina Paria et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Asfotase-α improves bone growth, mineralization and strength in mouse models of neurofibromatosis type-1

(2014) Jean de la Croix Ndong et al.   NATURE MEDICINE

Phase II study of sorafenib in children with recurrent or progressive low-grade astrocytomas

(2014) M. A. Karajannis et al.   NEURO-ONCOLOGY

Ras-Mek-Erk Signaling Regulates Nf1 Heterozygous Neointima Formation

(2013) Brian K. Stansfield et al.   AMERICAN JOURNAL OF PATHOLOGY

Hyperactive Ras/MAPK signaling is critical for tibial nonunion fracture in neurofibromin-deficient mice

(2013) Richa Sharma et al.   HUMAN MOLECULAR GENETICS

Approaches to Treating NF1 Tibial Pseudarthrosis

(2013) David A. Stevenson et al.   JOURNAL OF PEDIATRIC ORTHOPAEDICS

NF1 Deletion Generates Multiple Subtypes of Soft-Tissue Sarcoma That Respond to MEK Inhibition

(2013) R. D. Dodd et al.   MOLECULAR CANCER THERAPEUTICS

Recommendations for imaging tumor response in neurofibromatosis clinical trials

(2013) E. Dombi et al.   NEUROLOGY

Sensitivity of Glioblastomas to Clinically Available MEK Inhibitors Is Defined by Neurofibromin 1 Deficiency

(2012) W. L. See et al.   CANCER RESEARCH

Merlin, a multi-suppressor from cell membrane to the nucleus

(2012) Lu Zhou et al.   FEBS LETTERS

MEK inhibition exhibits efficacy in human and mouse neurofibromatosis tumors

(2012) Walter J. Jessen et al.   JOURNAL OF CLINICAL INVESTIGATION

Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2

(2011) Jaishri O. Blakeley et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

JNK inhibitors increase osteogenesis in Nf1-deficient cells

(2011) Kate Sullivan et al.   BONE

A murine model of neurofibromatosis type 1 tibial pseudarthrosis featuring proliferative fibrous tissue and osteoclast-like cells

(2011) Jad El-Hoss et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Control of proliferation in astrocytoma cells by the receptor tyrosine kinase/PI3K/AKT signaling axis and the use of PI-103 and TCN as potential anti-astrocytoma therapies

(2011) D. B. Gursel et al.   NEURO-ONCOLOGY

Erk1 Positively Regulates Osteoclast Differentiation and Bone Resorptive Activity

(2011) Yongzheng He et al.   PLoS One

Birth incidence and prevalence of tumor-prone syndromes: Estimates from a UK family genetic register service

(2010) D.G. Evans et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Integrated Genomic Analysis Identifies Clinically Relevant Subtypes of Glioblastoma Characterized by Abnormalities in PDGFRA, IDH1, EGFR, and NF1

(2010) Roel G.W. Verhaak et al.   CANCER CELL

Merlin, a “Magic” Linker Between the Extracellular Cues and Intracellular Signaling Pathways that Regulate Cell Motility, Proliferation, and Survival

(2010) Ivan Stamenkovic et al.   CURRENT PROTEIN & PEPTIDE SCIENCE

Skeletal abnormalities in neurofibromatosis type 1: Approaches to therapeutic options

(2009) Florent Elefteriou et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A