Submission Details

Submitter:

Classification:
Limited
GENCC:100004
Gene:
Disease:
Noonan syndrome with multiple lentigines
Mode Of Inheritance:
Autosomal dominant
Evaluated Date:
07/25/2018
Evidence/Notes:

There was only sufficient evidence in the literature for the association between RAF1 and Noonan syndrome with multiple lentigines (NSML) association to be classified as Limited. Only two variants with supporting evidence for pathogenicity (one de novo) in this gene have been found in individuals with NSML (Carcavilla et al., 2013; Kuburovic, Vukomanovic, Carcavilla, Ezquieta-Zubicaray, & Kuburovic, 2011; Pandit et al., 2007). The RAF1 gene is also located in the Ras/MAPK pathway, which is associated with the NSML phenotype (Aoki et al., 2016; Rauen, 2013). Of note, RAF1 is also classified as Definitive in association with Noonan syndrome (NS) and as Disputed in association with Costello syndrome and cardiofaciocutaneous syndrome. The ClinGen RASopathy Expert Panel found no evidence associating RAF1 with NS-like disorder with loose anagen hair. This curation was approved by the ClinGen RASopathy Gene Curation Expert Panel on 7/25/18 (SOP Version 5).

PubMed IDs:
17603483 20052757 21440552 22389993 23875798 24775816
Public Report:
Assertion Criteria:
Submitter Submitted Date:
12/05/2025

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