A decreased incidence of neuroblastomas in Down's syndrome and overproduction of S-100 b protein.
Neuroblastoma, one of the most frequent solid tumors found in childhood, is very rare in Down's syndrome subjects. This lack could possibly be due to overproduction of S-100 b protein for the following reasons: 1) the gene coding for S-100 b protein is situated on chromosome 21, and the protein is overproduced via a gene dosage effect; 2) S-100 b protein is found in glial cells and Schwann cells of the central and peripheral nervous system and has been shown to have a differentiating effect on normal neural cells; 3) neuroblastomas with a stroma rich in S-100 protein have a good prognosis. Preliminary studies demonstrated an inhibition of growth of two human neuroblastoma cell lines in the presence of S-100 b protein compared to controls. It is postulated that S-100 b protein may inhibit the development of neuroblastomas in Down's syndrome either antenatally, or after birth and may be a therapeutic agent against neuroblastoma.
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Laboratoire d'Anatomie Pathologique, Centre Hospitalier, Tulle, France.
D002455:Cell Division / Q000187:drug effects; D002648:Child; D004314:Down Syndrome / Q000150:complications* / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008954:Models, Biological; D009414:Nerve Growth Factors; D009447:Neuroblastoma / Q000150:complications* / Q000378:metabolism / Q000517:prevention & control; D011379:Prognosis; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins / Q000096:biosynthesis* / Q000235:genetics / Q000494:pharmacology; D014407:Tumor Cells, Cultured
D009414:Nerve Growth Factors; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins