Four frameshift mutations in neurofibromatosis type 1 caused by small insertions.

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Four frameshift mutations in neurofibromatosis type 1 caused by small insertions.

We have been using heteroduplex analysis to assay individual exons within the NF1 gene in an effort to identify disease causing constitutional mutations in neurofibromatosis type 1 patients. Here we report the identification and characterisation of four insertional NF1 frameshift mutations in an analysis of exons 28-39 in a set of 78 patients. These include three 1 base pair insertions and one ...

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SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotype.

OBJECTIVE Germline loss-of-function mutations in the SPRED1 gene have recently been identified in patients fulfilling the National Institutes of Health (NIH) diagnostic criteria for neurofibromatosis type 1 (NF1) but with no NF1 (neurofibromin 1) mutation found, suggesting a neurofibromatosis type 1-like syndrome. METHODS 61 index cases with NF1 clinical diagnosis but no identifiable NF1 muta...

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A Novel Frameshift Mutation in Neurofibromin 1 Gene in a Chinese Family with Neurofibromatosis Type 1

Correspondence To the Editor: Neurofibromatosis type 1 (NF1) is a common autosomal dominant genetic neurocutaneous disorder mainly characterized by café-au-lait macules (CALMs), neurofibromas, skinfold freckling, and Lisch nodules. [1] Mutations in the neurofibromin 1 gene (NF1) are known to solely result in NF1. [1] Most NF1 patients with mutations in NF1 are sporadic cases; the mean proportio...

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Neurofibromatosis type 1 is an autosomal dominantly inherited disease predisposing to a multitude of tumors, most characteristically benign plexiform neurofibromas and diffuse cutaneous neurofibromas. We investigated the presence and distribution of somatic mitochondrial DNA (mtDNA) mutations in neurofibromas and in nontumor tissue of neurofibromatosis type 1 patients. MtDNA alterations in the ...

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Genetic analysis of Neurofibromatosis type 1 (NF1) may facilitate the identification of patients in early phases of the disease. Here, we present an overview of our diagnostic research spanning the last 11 years, with a focus on the description of 225 NF1 mutations, 126 of which are novel, found in a series of 607 patients (513 unrelated) in Italy. Between 2003 and 2013, 443 unrelated patients ...

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 1997

ISSN: 1468-6244

DOI: 10.1136/jmg.34.7.579