Search Results - "Houten, Sander M"

The lysine degradation pathway: Subcellular compartmentalization and enzyme deficiencies by Leandro, João, Houten, Sander M.

“…Lysine degradation via formation of saccharopine is a pathway confined to the mitochondria. The second pathway for lysine degradation, the pipecolic acid…”
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Multilayered genetic and omics dissection of mitochondrial activity in a mouse reference population by Wu, Yibo, Williams, Evan G, Dubuis, Sébastien, Mottis, Adrienne, Jovaisaite, Virginija, Houten, Sander M, Argmann, Carmen A, Faridi, Pouya, Wolski, Witold, Kutalik, Zoltán, Zamboni, Nicola, Auwerx, Johan, Aebersold, Ruedi

“…The manner by which genotype and environment affect complex phenotypes is one of the fundamental questions in biology. In this study, we quantified the…”
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A Next Generation Multiscale View of Inborn Errors of Metabolism by Argmann, Carmen A., Houten, Sander M., Zhu, Jun, Schadt, Eric E.

“…Inborn errors of metabolism (IEM) are not unlike common diseases. They often present as a spectrum of disease phenotypes that correlates poorly with the…”
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Saccharopine, a lysine degradation intermediate, is a mitochondrial toxin by Leandro, João, Houten, Sander M

“…Saccharopine, a nonproteinogenic amino acid originally isolated from the yeast , is an intermediate in lysine metabolism. In this issue, Zhou et al. (2019…”
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Peroxisomes can oxidize medium‐ and long‐chain fatty acids through a pathway involving ABCD3 and HSD17B4 by Violante, Sara, Achetib, Nihad, Roermund, Carlo W. T., Hagen, Jacob, Dodatko, Tetyana, Vaz, Frédéric M., Waterham, Hans R., Chen, Hongjie, Baes, Myriam, Yu, Chunli, Argmann, Carmen A., Houten, Sander M.

“…ABSTRACT Peroxisomes are essential organelles for the specialized oxidation of a wide variety of fatty acids, but they are also able to degrade fatty acids…”
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Malnutrition-associated liver steatosis and ATP depletion is caused by peroxisomal and mitochondrial dysfunction by van Zutphen, Tim, Ciapaite, Jolita, Bloks, Vincent W, Ackereley, Cameron, Gerding, Albert, Jurdzinski, Angelika, de Moraes, Roberta Allgayer, Zhang, Ling, Wolters, Justina C, Bischoff, Rainer, Wanders, Ronald J, Houten, Sander M, Bronte-Tinkew, Dana, Shatseva, Tatiana, Lewis, Gary F, Groen, Albert K, Reijngoud, Dirk-Jan, Bakker, Barbara M, Jonker, Johan W, Kim, Peter K, Bandsma, Robert H.J

“…Graphical abstract…”
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SUMOylation-Dependent LRH-1/PROX1 Interaction Promotes Atherosclerosis by Decreasing Hepatic Reverse Cholesterol Transport by Stein, Sokrates, Oosterveer, Maaike H., Mataki, Chikage, Xu, Pan, Lemos, Vera, Havinga, Rick, Dittner, Claudia, Ryu, Dongryeol, Menzies, Keir J., Wang, Xu, Perino, Alessia, Houten, Sander M., Melchior, Frauke, Schoonjans, Kristina

“…Reverse cholesterol transport (RCT) is an antiatherogenic process in which excessive cholesterol from peripheral tissues is transported to the liver and…”
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Specific SIRT1 Activation Mimics Low Energy Levels and Protects against Diet-Induced Metabolic Disorders by Enhancing Fat Oxidation by Feige, Jérôme N., Lagouge, Marie, Canto, Carles, Strehle, Axelle, Houten, Sander M., Milne, Jill C., Lambert, Philip D., Mataki, Chikage, Elliott, Peter J., Auwerx, Johan

“…The NAD +-dependent deacetylase SIRT1 controls metabolic processes in response to low nutrient availability. We report the metabolic phenotype of mice treated…”
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PPARdelta activation induces metabolic and contractile maturation of human pluripotent stem cell-derived cardiomyocytes by Wickramasinghe, Nadeera M., Sachs, David, Shewale, Bhavana, Gonzalez, David M., Dhanan-Krishnan, Priyanka, Torre, Denis, LaMarca, Elizabeth, Raimo, Serena, Dariolli, Rafael, Serasinghe, Madhavika N., Mayourian, Joshua, Sebra, Robert, Beaumont, Kristin, Iyengar, Srinivas, French, Deborah L., Hansen, Arne, Eschenhagen, Thomas, Chipuk, Jerry E., Sobie, Eric A., Jacobs, Adam, Akbarian, Schahram, Ischiropoulos, Harry, Ma’ayan, Avi, Houten, Sander M., Costa, Kevin, Dubois, Nicole C.

“…Pluripotent stem-cell-derived cardiomyocytes (PSC-CMs) provide an unprecedented opportunity to study human heart development and disease, but they are…”
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PARP-2 Regulates SIRT1 Expression and Whole-Body Energy Expenditure by Bai, Péter, Canto, Carles, Brunyánszki, Attila, Huber, Aline, Szántó, Magdolna, Cen, Yana, Yamamoto, Hiroyasu, Houten, Sander M., Kiss, Borbala, Oudart, Hugues, Gergely, Pál, Menissier-de Murcia, Josiane, Schreiber, Valérie, Sauve, Anthony A., Auwerx, Johan

“…SIRT1 is a NAD+-dependent enzyme that affects metabolism by deacetylating key transcriptional regulators of energy expenditure. Here, we tested whether…”
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Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation by Pougovkina, Olga, te Brinke, Heleen, Ofman, Rob, van Cruchten, Arno G, Kulik, Wim, Wanders, Ronald J A, Houten, Sander M, de Boer, Vincent C J

“…Mitochondria integrate metabolic networks for maintaining bioenergetic requirements. Deregulation of mitochondrial metabolic networks can lead to mitochondrial…”
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Murine deficiency of peroxisomal l-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis by Ranea-Robles, Pablo, Violante, Sara, Argmann, Carmen, Dodatko, Tetyana, Bhattacharya, Dipankar, Chen, Hongjie, Yu, Chunli, Friedman, Scott L., Puchowicz, Michelle, Houten, Sander M.

“…Peroxisomes play an essential role in the β-oxidation of dicarboxylic acids (DCAs), which are metabolites formed upon ω-oxidation of fatty acids. Genetic…”
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Biallelic Mutations in MRPS34 Lead to Instability of the Small Mitoribosomal Subunit and Leigh Syndrome by Lake, Nicole J., Webb, Bryn D., Stroud, David A., Richman, Tara R., Ruzzenente, Benedetta, Compton, Alison G., Mountford, Hayley S., Pulman, Juliette, Zangarelli, Coralie, Rio, Marlene, Boddaert, Nathalie, Assouline, Zahra, Sherpa, Mingma D., Schadt, Eric E., Houten, Sander M., Byrnes, James, McCormick, Elizabeth M., Zolkipli-Cunningham, Zarazuela, Haude, Katrina, Zhang, Zhancheng, Retterer, Kyle, Bai, Renkui, Calvo, Sarah E., Mootha, Vamsi K., Christodoulou, John, Rötig, Agnes, Filipovska, Aleksandra, Cristian, Ingrid, Falk, Marni J., Metodiev, Metodi D., Thorburn, David R.

“…The synthesis of all 13 mitochondrial DNA (mtDNA)-encoded protein subunits of the human oxidative phosphorylation (OXPHOS) system is carried out by…”
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Adaptive reciprocity of lipid and glucose metabolism in human short-term starvation by Soeters, Maarten R, Soeters, Peter B, Schooneman, Marieke G, Houten, Sander M, Romijn, Johannes A

“…The human organism has tools to cope with metabolic challenges like starvation that are crucial for survival. Lipolysis, lipid oxidation, ketone body…”
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Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids by Houten, Sander M., Denis, Simone, Argmann, Carmen A., Jia, Yuzhi, Ferdinandusse, Sacha, Reddy, Janardan K., Wanders, Ronald J.A.

“…L-bifunctional enzyme (Ehhadh) is part of the classical peroxisomal fatty acid β-oxidation pathway. This pathway is highly inducible via peroxisome…”
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The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis by Ranea‐Robles, Pablo, Chen, Hongjie, Stauffer, Brandon, Yu, Chunli, Bhattacharya, Dipankar, Friedman, Scott L., Puchowicz, Michelle, Houten, Sander M.

“…Peroxisomes metabolize a specific subset of fatty acids, which include dicarboxylic fatty acids (DCAs) generated by ω‐oxidation. Data obtained in vitro suggest…”
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A novel deleterious ETFA promoter variant causative of multiple acyl‐CoA dehydrogenase deficiency by Prasun, Pankaj, Evans, Anthony, Cork, Emalyn, Houten, Sander M., Webb, Bryn D.

“…Multiple acyl‐CoA dehydrogenase deficiency (MADD) is an autosomal recessive disorder of fatty acid, amino acid, and choline metabolism. We describe a patient…”
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An integrative multiomic network model links lipid metabolism to glucose regulation in coronary artery disease by Cohain, Ariella T., Barrington, William T., Jordan, Daniel M., Beckmann, Noam D., Argmann, Carmen A., Houten, Sander M., Charney, Alexander W., Ermel, Raili, Sukhavasi, Katyayani, Franzen, Oscar, Koplev, Simon, Whatling, Carl, Belbin, Gillian M., Yang, Jialiang, Hao, Ke, Kenny, Eimear E., Tu, Zhidong, Zhu, Jun, Gan, Li-Ming, Do, Ron, Giannarelli, Chiara, Kovacic, Jason C., Ruusalepp, Arno, Lusis, Aldons J., Bjorkegren, Johan L. M., Schadt, Eric E.

“…Elevated plasma cholesterol and type 2 diabetes (T2D) are associated with coronary artery disease (CAD). Individuals treated with cholesterol-lowering statins…”
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Changes in the Metabolome in Response to Low-Dose Exposure to Environmental Chemicals Used in Personal Care Products during Different Windows of Susceptibility by Houten, Sander M, Chen, Jia, Belpoggi, Fiorella, Manservisi, Fabiana, Sánchez-Guijo, Alberto, Wudy, Stefan A, Teitelbaum, Susan L

“…The consequences of ubiquitous exposure to environmental chemicals remain poorly defined. Non-targeted metabolomic profiling is an emerging method to identify…”
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Acyl-CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism by Houten, Sander M, Dodatko, Tetyana, Dwyer, William, Violante, Sara, Chen, Hongjie, Stauffer, Brandon, DeVita, Robert J, Vaz, Frédéric M, Cross, Justin R, Yu, Chunli, Leandro, João

“…Toxicity of accumulating substrates is a significant problem in several disorders of valine and isoleucine degradation notably short-chain enoyl-CoA hydratase…”
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