Results 111 to 120 of about 25,840 (207)

Outcomes following definitive treatment of malignant peripheral nerve sheath tumor are significantly worse for patients with neurofibromatosis type 1: A Canadian Sarcoma Research and Clinical Collaboration study

open access: yesCancer, Volume 132, Issue 3, 1 February 2026.
Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with a 5‐year survival rate of approximately 50%, secondary to their metastatic potential and resistance to therapy. MPNSTs can arise sporadically, as a late toxicity from therapeutic radiotherapy, or in patients with neurofibromatosis type 1 (NF1).
Rachel E. Aubrey   +7 more
wiley   +1 more source

Recent Advancement of Neurofibromatosis Type 1: A Narrative Review

open access: yesActa Neurologica Taiwanica
Neurofibromatosis type 1 (NF1) is a complex autosomal dominant genetic disorder caused by mutations in the NF1 gene on chromosome 17, which encodes the tumor-suppressor protein, neurofibromin.
Po-Yuan Huang   +2 more
doaj   +1 more source

Tranilast inhibits the expression of genes related to epithelial-mesenchymal transition and angiogenesis in neurofibromin-deficient cells

open access: yesScientific Reports, 2018
Neurofibromatosis type 1 (NF1) is caused by germline mutations in the NF1 gene and is characterized by café au lait spots and benign tumours known as neurofibromas.
Ritsuko Harigai   +13 more
doaj   +1 more source

Clinically aggressive central giant cell granulomas in two patients with neurofibromatosis 1 [PDF]

open access: yes, 2006
Background Neurofibromatosis 1 (NF1) is an autosomal dominantly inherited disorder caused by a spectrum of mutations affecting the Nf1 gene. Affected patients develop benign and malignant tumors at an increased frequency.
Edwards, Paul C.   +5 more
core   +1 more source

Clinical exome performance for reporting secondary genetic findings. [PDF]

open access: yes, 2014
BACKGROUND : Reporting clinically actionable incidental genetic findings in the course of clinical exome testing is recommended by the American College of Medical Genet- ics and Genomics (ACMG).
Clark, P   +6 more
core   +1 more source

Alphavirus production is inhibited in neurofibromin 1-deficient cells through activated RAS signalling

open access: yesVirology, 2008
Virus-host interactions essential for alphavirus pathogenesis are poorly understood. To address this shortcoming, we coupled retrovirus insertional mutagenesis and a cell survival selection strategy to generate clonal cell lines broadly resistant to Sindbis virus (SINV) and other alphaviruses.
Kolokoltsova, Olga A.   +5 more
openaire   +2 more sources

A neurofibromatosis type 1 family report with multiple cases in 3 consecutive Generations

open access: yesمجله دانشگاه علوم پزشکی گرگان, 2017
Neurofibromatosis type1 (NF1) with the incidence of 1 in 3500 births, is the most common disorder which affects skin and peripheral nervous system. NF1 results from mutations in NF1 gene. The NF1 gene spans 350kbp and to date, nearly 2434 mutations in it
M Oladnabi   +4 more
doaj  

Preclinical Evidence for the Use of Sunitinib Malate in the Treatment of Plexiform Neurofibromas [PDF]

open access: yes, 2015
Plexiform neurofibromas (pNF) are pathognomonic nerve and soft tissue tumors of neurofibromatosis type I (NF1), which are highly resistant to conventional chemotherapy and associated with significant morbidity/mortality.
Chen, Shi   +13 more
core   +1 more source

Nf1 haploinsufficiency alters myeloid lineage commitment and function, leading to deranged skeletal homeostasis [PDF]

open access: yes, 2015
Although nullizygous loss of NF1 leads to myeloid malignancies, haploinsufficient loss of NF1 (Nf1) has been shown to contribute to osteopenia and osteoporosis which occurs in approximately 50% of neurofibromatosis type 1 (NF1) patients.
Chen, Shi   +8 more
core   +1 more source

Diagnostic and therapeutic process of neurofibromatosis type 1 and type 2

open access: yesJournal of Education, Health and Sport
Neurofibromatosis is one of the most common genetic diseases. It is inherited in an autosomal dominant manner. It is divided into two genetically distinct subtypes, characterised by multiple skin lesions and tumours of the peripheral and central nervous 
Michał Leśniewski   +3 more
doaj   +1 more source

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