[HTML][HTML] Neurofibromatosis 1

JM Friedman - 2022 - europepmc.org
Neurofibromatosis 1 (NF1) is a multisystem disorder characterized by multiple café au lait
macules, intertriginous freckling, multiple cutaneous neurofibromas, and learning disability …

Uncovering novel mutational signatures by de novo extraction with SigProfilerExtractor

SMA Islam, M Díaz-Gay, Y Wu, M Barnes, R Vangara… - Cell genomics, 2022 - cell.com
Mutational signature analysis is commonly performed in cancer genomic studies. Here, we
present SigProfilerExtractor, an automated tool for de novo extraction of mutational …

Cutaneous neurofibromas in the genomics era: current understanding and open questions

RJ Allaway, SJC Gosline, S La Rosa, P Knight… - British journal of …, 2018 - nature.com
Cutaneous neurofibromas (cNF) are a nearly ubiquitous symptom of neurofibromatosis type
1 (NF1), a disorder with a broad phenotypic spectrum caused by germline mutation of the …

Whole Exome Sequencing Identifies TSC1/TSC2 Biallelic Loss as the Primary and Sufficient Driver Event for Renal Angiomyolipoma Development

K Giannikou, IA Malinowska, TJ Pugh, R Yan… - PLoS …, 2016 - journals.plos.org
Renal angiomyolipoma is a kidney tumor in the perivascular epithelioid (PEComa) family
that is common in patients with Tuberous Sclerosis Complex (TSC) and …

[HTML][HTML] Immortalization of human normal and NF1 neurofibroma Schwann cells

H Li, LJ Chang, DR Neubauer, DF Muir… - Laboratory …, 2016 - Elsevier
Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the
autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic …

Recent advances in the diagnosis and pathogenesis of neurofibromatosis type 1 (NF1)-associated peripheral nervous system neoplasms

JF Longo, SM Weber, BP Turner-Ivey… - Advances in anatomic …, 2018 - journals.lww.com
The diagnosis of a neurofibroma or a malignant peripheral nerve sheath tumor (MPNST)
often raises the question of whether the patient has the genetic disorder neurofibromatosis …

[HTML][HTML] Characterization of immune cell populations of cutaneous neurofibromas in neurofibromatosis 1

RA Kallionpää, S Peltonen, KM Le, E Martikkala… - Laboratory …, 2024 - Elsevier
Cutaneous neurofibromas (cNFs) are characteristic of neurofibromatosis 1 (NF1), yet their
immune microenvironment is incompletely known. A total of 61 cNFs from 10 patients with …

NF1 patient missense variants predict a role for ATM in modifying neurofibroma initiation

Y Yu, K Choi, J Wu, PR Andreassen, PJ Dexheimer… - Acta …, 2020 - Springer
In Neurofibromatosis type 1, NF1 gene mutations in Schwann cells (SC) drive benign
plexiform neurofibroma (PNF), and no additional SC changes explain patient-to-patient …

Computational insights of K1444N substitution in GAP-related domain of NF1 gene associated with neurofibromatosis type 1 disease: a molecular modeling and …

AK Agrahari, M Muskan, C George Priya Doss… - Metabolic Brain …, 2018 - Springer
The NF1 gene encodes for neurofibromin protein, which is ubiquitously expressed, but most
highly in the central nervous system. Non-synonymous SNPs (nsSNPs) in the NF1 gene …

Contemporary Approach to Neurofibromatosis Type 1–Associated Malignant Peripheral Nerve Sheath Tumors

AC Hirbe, CA Dehner, E Dombi, V Eulo… - American Society of …, 2024 - ascopubs.org
Most malignant peripheral nerve sheath tumors (MPNSTs) are clinically aggressive high-
grade sarcomas, arising in individuals with neurofibromatosis type 1 (NF1) at a significantly …