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LINKAGE ANALYSIS IN DUTCH FAMILIAL ATYPICAL MULTIPLE MOLE MELANOMA (FAMMM) SYNDROME FAMILIES - EFFECT OF NEVUS COUNT
Author(s): GRUIS NA, SANDKUIJL LA, WEBER JL, VANDERZEE A, BORGSTEIN AM, BERGMAN W, FRANTS RR
Source: MELANOMA RESEARCH    Volume: 3    Issue: 4    Pages: 271-277    Published: AUG 1993  
Times Cited: 52     References: 0     
Abstract: Familial atypical multiple mole-melanoma (FAMMM) syndrome is characterized by the familial occurrence of malignant melanoma of the skin in combination with multiple atypical precursor naevi. In the present study we performed linkage analysis in seven Dutch FAMMM families to define the relationship between the ultimate phenotype melanoma and the postulated precursors, atypical (dysplastic) naevi. Various models were defined, varying from melanoma only to various combinations of melanoma and atypical naevi, reflecting the FAMMM phenotype. Using 124 microsatellite markers spread across all autosomes, hints for linkage were obtained between several chromosome 9p markers and a melanoma locus (D9S171; odds for linkage, 275:1). In a model including melanoma and a florid manifestation of atypical naevi a considerably higher lod score was obtained with D9S171 (odds for linkage, 4365:1); models including milder manifestations yielded less support. We conclude that, also in the Dutch FAMMM families, a melanoma gene is located on the short arm of chromosome 9 and that multiple atypical naevi, at least in certain cases, seems to be a component of the FAMMM phenotype.
Document Type: Proceedings Paper
Language: English
Addresses:
1. LEIDEN UNIV, DEPT HUMAN GENET, MGC, POB 9503, 2300 RA LEIDEN, NETHERLANDS
Publisher: RAPID SCIENCE PUBLISHERS, 2-6 BOUNDARY ROW, LONDON, ENGLAND SE1 8NH
Subject Category: Oncology; Dermatology; Medicine, Research & Experimental
IDS Number: LZ178
ISSN: 0960-8931
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