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PMID: 7203480 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

Retinoblastoma: host resistance and 13q- chromosomal deletion.

Human genetics ·Vol. 56 ·No. 1 ·1980-00-00 ·Pages 53-8

Matsunaga E

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.

MeSH Terms
Alleles Child Chromosome Deletion Chromosomes, Human, 13-15 Eye Neoplasms/genetics,pathology Functional Laterality Genes Humans Phenotype Retinoblastoma/genetics,pathology Suppression, Genetic
Authors & Affiliations
1 authors, click to expand affiliations / ORCID
Matsunaga E
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34 references, click to expand
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Article Info
Journal
Human genetics
Abbr.
Hum Genet
ISSN
0340-6717
Published
1980-00-00
Pages
53-8
Language
English
Region
Germany
NLM ID
7613873
Subset
IM
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