Evidence of chromosomal instability in the lymphocytes of Gorlin basal-cell carcinoma patients.
The Gorlin syndrome, or naevoid basal-cell carcinoma syndrome (NBCS) is an autosomal dominant disease. It has been suspected for long that this cancer prone disease (multiple basal-cell carcinomas; other malignant or benign proliferations) is a chromosome instability syndrome. We previously reported a lengthening in the cell cycle of lymphocytes from two patients with NBCS. With a larger sample (n = 7), we confirm this disease to be a chromosome instability syndrome, although clearly, expression of this characteristic can vary between patients: (1) spontaneous chromatid breaks occurred more often in a subset of the patients; (2) spontaneous micronuclei were found more frequently in NBCS than in the controls; (3) we confirm the cell cycle to be affected in this disease. As these results were obtained on lymphocytes--a cell lineage not affected in NBCS manifestations--the chromosome instability we found would appear to be part of the general condition of this syndrome.
Shafei-Benaissa
E
E
Cytogénétique, Laboratoire Hématologie, CHU la Milètrie, Poitiers, France.
Savage
J R
JR
Papworth
D
D
Babin
P
P
Larrègue
M
M
Tanzer
J
J
Bonnetblanc
J M
JM
Vaillant
L
L
Huret
J L
JL
D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000235:genetics* / Q000473:pathology; D002453:Cell Cycle / Q000235:genetics; D002869:Chromosome Aberrations; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000473:pathology* / Q000648:ultrastructure; D008297:Male; D015162:Micronucleus Tests; D008875:Middle Aged; D012854:Sister Chromatid Exchange