Evidence of apoptosis and mitochondrial abnormalities in peripheral blood cells of Huntington's disease patients.
The mechanisms by which neurons die in Huntington's disease (HD) are uncertain, however, mitochondrial dysfunction and apoptosis have been implicated. Because peripheral abnormalities may reflect similar consequences of mutant huntingtin in the brain, we evaluated markers of apoptotic cell death and mitochondrial function in peripheral blood cells of 10 HD patients and 16 age- and gender-matched controls. We found increased Bax expression in B and T lymphocytes, and monocytes from HD patients, but no alterations in Bcl-2 expression levels. B lymphocytes also showed decreased mitochondrial membrane potential. However, HD peripheral blood cells showed no differences in reactive oxygen species (ROS) levels when compared to controls. Our results suggest that peripheral blood cells, in particularly B lymphocytes may reflect changes observed in HD brain.
Almeida
Sandra
S
Center for Neuroscience and Cell Biology of Coimbra, University of Coimbra, 3004-504 Coimbra, Portugal.
Sarmento-Ribeiro
Ana Bela
AB
Januário
Cristina
C
Rego
A Cristina
AC
Oliveira
Catarina R
CR
D017209:Apoptosis; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000378:metabolism / Q000473:pathology*; D007962:Leukocytes / Q000378:metabolism / Q000648:ultrastructure*; D008214:Lymphocytes / Q000378:metabolism / Q000648:ultrastructure; D008297:Male; D008928:Mitochondria / Q000378:metabolism / Q000473:pathology*; D009000:Monocytes / Q000378:metabolism / Q000648:ultrastructure; D009504:Neutrophils / Q000378:metabolism / Q000648:ultrastructure; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D051028:bcl-2-Associated X Protein / Q000378:metabolism
D019253:Proto-Oncogene Proteins c-bcl-2; D051028:bcl-2-Associated X Protein