Genetic variation in PARL influences mitochondrial content

Given their involvement in processes necessary for life, mitochondrial damage and subsequent dysfunction can lead to a wide range of human diseases. Previous studies of both animal models and humans have suggested that presenilins-associated rhomboid-like protein (PARL) is a key regulator of mitocho...

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Main Authors: Curran, J., Jowett, J., Abraham, L., Diepeveen, L., Elliott, K., Dyer, T., Kerr-Bayles, L., Johnson, M., Comuzzie, A., Moses, Eric, Walder, K., Collier, G., Blangero, J., Kissebah, A.
Format: Journal Article
Published: 2010
Online Access:http://hdl.handle.net/20.500.11937/7519
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author Curran, J.
Jowett, J.
Abraham, L.
Diepeveen, L.
Elliott, K.
Dyer, T.
Kerr-Bayles, L.
Johnson, M.
Comuzzie, A.
Moses, Eric
Walder, K.
Collier, G.
Blangero, J.
Kissebah, A.
author_facet Curran, J.
Jowett, J.
Abraham, L.
Diepeveen, L.
Elliott, K.
Dyer, T.
Kerr-Bayles, L.
Johnson, M.
Comuzzie, A.
Moses, Eric
Walder, K.
Collier, G.
Blangero, J.
Kissebah, A.
author_sort Curran, J.
building Curtin Institutional Repository
collection Online Access
description Given their involvement in processes necessary for life, mitochondrial damage and subsequent dysfunction can lead to a wide range of human diseases. Previous studies of both animal models and humans have suggested that presenilins-associated rhomboid-like protein (PARL) is a key regulator of mitochondrial integrity and function, and plays a role in cellular apoptosis. As a surrogate measure of mitochondrial integrity, we previously measured mitochondrial content in a Caucasian population consisting of large extended pedigrees, with results highlighting a substantial genetic component to this trait. To assess the influence of variation in the PARL gene on mitochondrial content, we re-sequenced 6.5 kb of the gene, identifying 16 SNPs and genotyped these in 1,086 Caucasian individuals, distributed across 170 families. Statistical genetic analysis revealed that one promoter variant, T-191C, exhibited significant effects (after correction for multiple testing) on mitochondrial content levels. Comparison of the transcription factor binding characteristics of the T-191C promoter SNP by EMSA indicates preferential binding of nuclear factors to the T allele, suggesting functional variation in PARL expression. These results suggest that genetic variation within PARL influences mitochondrial abundance and integrity. © 2009 Springer-Verlag.
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spelling curtin-20.500.11937-75192018-03-29T09:05:39Z Genetic variation in PARL influences mitochondrial content Curran, J. Jowett, J. Abraham, L. Diepeveen, L. Elliott, K. Dyer, T. Kerr-Bayles, L. Johnson, M. Comuzzie, A. Moses, Eric Walder, K. Collier, G. Blangero, J. Kissebah, A. Given their involvement in processes necessary for life, mitochondrial damage and subsequent dysfunction can lead to a wide range of human diseases. Previous studies of both animal models and humans have suggested that presenilins-associated rhomboid-like protein (PARL) is a key regulator of mitochondrial integrity and function, and plays a role in cellular apoptosis. As a surrogate measure of mitochondrial integrity, we previously measured mitochondrial content in a Caucasian population consisting of large extended pedigrees, with results highlighting a substantial genetic component to this trait. To assess the influence of variation in the PARL gene on mitochondrial content, we re-sequenced 6.5 kb of the gene, identifying 16 SNPs and genotyped these in 1,086 Caucasian individuals, distributed across 170 families. Statistical genetic analysis revealed that one promoter variant, T-191C, exhibited significant effects (after correction for multiple testing) on mitochondrial content levels. Comparison of the transcription factor binding characteristics of the T-191C promoter SNP by EMSA indicates preferential binding of nuclear factors to the T allele, suggesting functional variation in PARL expression. These results suggest that genetic variation within PARL influences mitochondrial abundance and integrity. © 2009 Springer-Verlag. 2010 Journal Article http://hdl.handle.net/20.500.11937/7519 10.1007/s00439-009-0756-0 restricted
spellingShingle Curran, J.
Jowett, J.
Abraham, L.
Diepeveen, L.
Elliott, K.
Dyer, T.
Kerr-Bayles, L.
Johnson, M.
Comuzzie, A.
Moses, Eric
Walder, K.
Collier, G.
Blangero, J.
Kissebah, A.
Genetic variation in PARL influences mitochondrial content
title Genetic variation in PARL influences mitochondrial content
title_full Genetic variation in PARL influences mitochondrial content
title_fullStr Genetic variation in PARL influences mitochondrial content
title_full_unstemmed Genetic variation in PARL influences mitochondrial content
title_short Genetic variation in PARL influences mitochondrial content
title_sort genetic variation in parl influences mitochondrial content
url http://hdl.handle.net/20.500.11937/7519