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Neurofibromatosis type 1 associated low grade gliomas: A comparison with sporadic low grade gliomas

Publication date: Available online 21 May 2016
Source:Critical Reviews in Oncology/Hematology
Author(s): Jelte Helfferich, Ronald Nijmeijer, Oebele F. Brouwer, Maartje Boon, Annemarie Fock, Eelco W. Hoving, Lisethe Meijer, Wilfred F.A. den Dunnen, Eveline S.J.M. de Bont
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder, associated with a variable clinical phenotype including café-au-lait spots, intertriginous freckling, Lisch nodules, neurofibromas, optic pathway gliomas and distinctive bony lesions. NF1 is caused by a mutation in the NF1 gene, which codes for neurofibromin, a large protein involved in the MAPK- and the mTOR-pathway through RAS-RAF signalling.NF1 is a known tumour predisposition syndrome, associated with different tumours of the nervous system including low grade gliomas (LGGs) in the paediatric population. The focus of this review is on grade I pilocytic astrocytomas (PAs), the most commonly observed histologic subtype of low grade gliomas in NF1. Clinically, these PAs have a better prognosis and show different localisation patterns than their sporadic counterparts, which are most commonly associated with a KIAA1549:BRAF fusion.In this review, possible mechanisms of tumourigenesis in LGGs with and without NF1 will be discussed, including the contribution of different signalling pathways and tumour microenvironment. Furthermore we will discuss how increased understanding of tumourigenesis may lead to new potential targets for treatment.



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The Armor of God (HUASTEC, VERACRUZ: Bible NT)

The Armor of God (HUASTEC, VERACRUZ: Bible NT)

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HUASTEC, VERACRUZ: Bible NT

Ephesians (Efesios) 6:10-18

10 Ani xo kidhblbtsik, ka likedhatsik tab abal i kwajatichtsik junax kal an Ajtiklb ani kal nin pulek chp xi Jaj in kool.

11 Jelt xan ti an soldadotsik in kool kidhat xowa in ynl ti pjx, anchan teye ti ka koytsik xowa in pidhnl na Dios abal ka ejto ki kwajaytsik alwa ani yab ki kambiyattsik kal an lej atax.

12Pos yab exom tu pjx kal i…

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“Dreamy Tree Photograph Whispers” / “Dogwood blossoms”, Catherine Jeltes “Never man invent nothing simpler or more beautiful than a manifestation of nature. Given the cause, nature produces the effect, the shortest way that can be produced. ”“, Leonardo de Vinci

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Neurofibromatosis type 1 associated low grade gliomas: A comparison with sporadic low grade gliomas

Publication date: Available online 21 May 2016Source:Critical Reviews in Oncology/Hematology
Author(s): Jelte Helfferich, Ronald Nijmeijer, Oebele F. Brouwer, Maartje Boon, Annemarie Fock, Eelco W. Hoving, Lisethe Meijer, Wilfred F.A. den Dunnen, Eveline S.J.M. de Bont
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder, associated with a variable clinical phenotype including café-au-lait spots, intertriginous freckling, Lisch nodules, neurofibromas, optic pathway gliomas and distinctive bony lesions. NF1 is caused by a mutation in the NF1 gene, which codes for neurofibromin, a large protein involved in the MAPK- and the mTOR-pathway through RAS-RAF signalling.NF1 is a known tumour predisposition syndrome, associated with different tumours of the nervous system including low grade gliomas (LGGs) in the paediatric population. The focus of this review is on grade I pilocytic astrocytomas (PAs), the most commonly observed histologic subtype of low grade gliomas in NF1. Clinically, these PAs have a better prognosis and show different localisation patterns than their sporadic counterparts, which are most commonly associated with a KIAA1549:BRAF fusion.In this review, possible mechanisms of tumourigenesis in LGGs with and without NF1 will be discussed, including the contribution of different signalling pathways and tumour microenvironment. Furthermore we will discuss how increased understanding of tumourigenesis may lead to new potential targets for treatment.
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