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Retinoblastoma: host resistance and 13q- chromosomal deletion.

Abstract：

:Data for 27 cases of retinoblastoma that developed in patients with 13q- were collected from the literature and analyzed. The distribution of unilateral and bilateral cases of retinoblastoma differed significantly from the expectation that the degree of expressivity does not differ between the retinoblastoma gene and deletion of 13q. The excess of unilateral cases among the patients with 13q-, which could not be accounted by ascertainment bias, was attributed to somewhat lowered carcinogenic potential of deletion of 13q14 as compared with the retinoblastoma gene. It was argued that the retinoblastoma gene is probably not located on 13q, and perhaps 20% or more of the individuals with a deletion of 13q14 would not develop retinoblastoma. The normal allele at the retinoblastoma locus, the haplicon in the segment of 13q14, and the suppressor genes as defined by the host resistance model, may be all concerned, in their function additively and without dominance, with normal differentiation of the embryonic retinal cells.

journal_name

Hum Genet

journal_title

Human genetics

authors

Matsunaga E

doi

10.1007/BF00281568

subject

Has Abstract

pub_date

1980-01-01 00:00:00

pages

53-8

issue

1

eissn

0340-6717

issn

1432-1203

journal_volume

56

pub_type

杂志文章

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