Idioma:

Runx1 contributes to neurofibromatosis type 1 neurofibroma formation

Li, H ; Zhao, X ; Yan, X ; Jessen, W J ; Kim, M-O ; Dombi, E ; Liu, P P ; Huang, G ; Wu, J

Oncogene, 2016-03, Vol.35 (11), p.1468-1474 [Periódico revisado por pares]

England: Nature Publishing Group

Texto completo disponível

Citações Citado por

Enviar para

Título:
Runx1 contributes to neurofibromatosis type 1 neurofibroma formation
Autor: Li, H ; Zhao, X ; Yan, X ; Jessen, W J ; Kim, M-O ; Dombi, E ; Liu, P P ; Huang, G ; Wu, J
Assuntos: Animals ; Carrier Proteins - metabolism ; CCAAT-Binding Factor - antagonists & inhibitors ; Cell Line, Tumor ; Cell Transformation, Neoplastic - pathology ; Comparative analysis ; Core Binding Factor Alpha 2 Subunit - biosynthesis ; Core Binding Factor Alpha 2 Subunit - genetics ; Core Binding Factor Alpha 2 Subunit - metabolism ; Development and progression ; DNA microarrays ; Embryos ; Fatty Acid-Binding Protein 7 ; Gene deletion ; Gene expression ; Genetic aspects ; Genetic disorders ; Health aspects ; Humans ; Mice ; mRNA ; Neurofibroma - pathology ; Neurofibroma, Plexiform - pathology ; Neurofibromatosis ; Neurofibromatosis 1 - pathology ; Neurofibromin 1 - genetics ; Neurological disorders ; Oncology ; Patients ; Progenitor cells ; Properties ; Protein expression ; Recklinghausen's disease ; RNA Interference ; RNA, Small Interfering - genetics ; Runx1 protein ; Schwann cells ; Schwann Cells - cytology ; Stem Cells - cytology ; Transcription factors ; Tumor Suppressor Proteins - metabolism ; Tumors
É parte de: Oncogene, 2016-03, Vol.35 (11), p.1468-1474
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
Descrição: Neurofibromatosis type 1 (NF1) patients are predisposed to neurofibromas but the driver(s) that contribute to neurofibroma formation are not fully understood. By cross comparison of microarray gene lists on human neurofibroma-initiating cells and developed neurofibroma Schwann cells (SCs) we identified RUNX1 overexpression in human neurofibroma initiation cells, suggesting RUNX1 might relate to neurofibroma formation. Immunostaining confirmed RUNX1 protein overexpression in human plexiform neurofibromas. Runx1 overexpression was confirmed in mouse Schwann cell progenitors (SCPs) and mouse neurofibromas at the messenger RNA and protein levels. Genetic inhibition of Runx1 expression by small hairpin RNA or pharmacological inhibition of Runx1 function by a Runx1/Cbfβ interaction inhibitor, Ro5-3335, decreased mouse neurofibroma sphere number in vitro. Targeted genetic deletion of Runx1 in SCs and SCPs delayed mouse neurofibroma formation in vivo. Mechanistically, loss of Nf1 increased embryonic day 12.5 Runx1(+)/Blbp(+) progenitors that enable tumor formation. These results suggest that Runx1 has an important role in Nf1 neurofibroma initiation, and inhibition of RUNX1 function might provide a novel potential therapeutic treatment strategy for neurofibroma patients.
Editor: England: Nature Publishing Group
Idioma: Inglês

Links

View this record in MEDLINE/PubMed

Voltar para lista de resultados

Realização: Logos de Redes Sociais:

Runx1 contributes to neurofibromatosis type 1 neurofibroma formation

Li, H ; Zhao, X ; Yan, X ; Jessen, W J ; Kim, M-O ; Dombi, E ; Liu, P P ; Huang, G ; Wu, J

England: Nature Publishing Group

Buscando em bases de dados remotas. Favor aguardar.