Telomerase activity in cell lines and lymphoma originating from Bloom syndrome.
Bloom syndrome (BS) is characterized by premature aging and high predisposition to various types of cancer. BLM is the causative gene for BS. BLM functions as a DNA helicase in the direction of 3' to 5' and small subsets of telomeres colocalize with BLM protein. We investigated telomerase activity and telomere repeat length in the cells from BS patients. In Epstein-Barr-virus (EBV) transformed lymphoblastoid cell lines and lymphoma cells from BS patients, telomerase activity was detected as in the control and compared. The metastatic tumor from BS patient, which had a 9-bp deletion of p53 DNA showed the strongest telomerase activity. Telomere repeat length in BS cells showed that there is no large difference compared with normal cells. Collectively, the results show that the BLM gene is not a major structural and regulatory factor in maintaining telomere repeat length and telomerase activity.
Kaneko
H
H
Department of Pediatrics, Gifu University School of Medicine, Japan. hideo@cc.gifu-u.ac.jp
Morimoto
W
W
Fukao
T
T
Kasahara
K
K
Kondo
N
N
D000251:Adenosine Triphosphatases / Q000235:genetics / Q000502:physiology; D000328:Adult; D001816:Bloom Syndrome / Q000150:complications / Q000201:enzymology* / Q000235:genetics; D002461:Cell Line, Transformed; D004265:DNA Helicases / Q000235:genetics / Q000502:physiology; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000201:enzymology* / Q000209:etiology / Q000473:pathology; D008297:Male; D053484:RecQ Helicases; D019098:Telomerase / Q000378:metabolism*; D016615:Telomere / Q000378:metabolism / Q000648:ultrastructure
D019098:Telomerase; D000251:Adenosine Triphosphatases; C097151:Bloom syndrome protein; D004265:DNA Helicases; D053484:RecQ Helicases