Basal Cell Nevus Syndrome

Known as: Gorlin Goltz Syndrome, Fifth Phacomatosis, gorlins syndrome 
An autosomal dominant genetic syndrome caused by abnormalities in the PTCH gene. It is characterized by multiple basal cell carcinomas at a young age… (More)
National Institutes of Health

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Topic mentions per year

1963-2017
020406019632017

Papers overview

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Highly Cited
2006
Highly Cited
2006
The Hedgehog (Hh) pathway plays important roles during embryogenesis and carcinogenesis. Here, we show that ablation of the mouse… (More)
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Highly Cited
2002
Highly Cited
2002
The sonic hedgehog (SHH) signaling pathway directs the embryonic development of diverse organisms and is disrupted in a variety… (More)
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Highly Cited
1998
Highly Cited
1998
Mutations in PATCHED (PTC), the human homolog of the Drosophila patched gene, have been identified in most exons of the gene in… (More)
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Highly Cited
1997
Highly Cited
1997
The PATCHED (PTC) gene encodes a Sonic hedgehog (Shh) receptor and a tumor suppressor protein that is defective in basal cell… (More)
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Highly Cited
1997
Highly Cited
1997
Mutations in the tumor suppressor gene PATCHED (PTC) are found in human patients with the basal cell nevus syndrome, a disease… (More)
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Highly Cited
1997
Highly Cited
1997
Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin's syndrome, is an autosomal dominant disorder that predisposes to… (More)
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Highly Cited
1997
Highly Cited
1997
Inactivating mutations in the PTCH gene, a human homologue of the Drosophila segment polarity gene patched, have been identified… (More)
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Highly Cited
1996
Highly Cited
1996
The basal cell nevus syndrome (BCNS) is characterized by developmental abnormalities and by the postnatal occurrence of cancers… (More)
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Highly Cited
1996
Highly Cited
1996
Basal cell carcinoma (BCC) is the most common cancer in humans. The majority of sporadic BCCs have allele loss on chromosome 9q22… (More)
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1986
1986
Six cases of familial basal cell nevus syndrome encountered over the past 20 years are described. The syndrome is fairly rare and… (More)
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