Document Detail

Telomere length in Hutchinson-Gilford progeria syndrome.
MedLine Citation:
PMID:  19428457     Owner:  NLM     Status:  MEDLINE    
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare premature aging disorder caused by mutations in the gene LMNA, which encodes the nuclear matrix protein lamin A. Previous research has shown that the average telomere length in fibroblasts from HGPS patients is shorter than in age-matched controls. How mutations in lamin A lead to shortened telomere lengths is not known nor is the contribution of individual chromosome ends to the low average length understood. To measure the telomere length of individual chromosomes, we used quantitative fluorescence in situ hybridization (Q-FISH). In agreement with previous studies, we found that the average telomere length in HPGS fibroblasts is greatly reduced; however, the telomere length at chromosome ends was variable. In contrast, the telomere length in hematopoietic cells which typically do not express lamin A, was within the normal range for three out of four HGPS patient samples. Our results suggest that mutant lamin A decreases telomere length via a direct effect and that expression of mutant LMNA is necessary for telomere loss in HGPS.
Michelle L Decker; Elizabeth Chavez; Irma Vulto; Peter M Lansdorp
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-03-20
Journal Detail:
Title:  Mechanisms of ageing and development     Volume:  130     ISSN:  1872-6216     ISO Abbreviation:  Mech. Ageing Dev.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-05-11     Completed Date:  2009-07-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0347227     Medline TA:  Mech Ageing Dev     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  377-83     Citation Subset:  IM    
Terry Fox Laboratory, British Columbia Cancer Agency, 675 West 10th Avenue, Vancouver, BC, Canada.
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MeSH Terms
Aged, 80 and over
Case-Control Studies
Cell Line
Child, Preschool
Fibroblasts / metabolism*
Granulocytes / metabolism
In Situ Hybridization, Fluorescence
Infant, Newborn
Lamin Type A / genetics*
Middle Aged
Progeria / genetics*
T-Lymphocytes / metabolism
Telomere / metabolism*
Young Adult
Reg. No./Substance:
0/Lamin Type A

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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