The burden of trisomy 21 disrupts the proteostasis network in Down syndrome von Aivazidis, Stefanos, Coughlan, Christina M, Rauniyar, Abhishek K, Jiang, Hua, Liggett, L Alexander, Maclean, Kenneth N, Roede, James R

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Variant non-ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5 von BAKER, Peter R, FRIEDERICH, Marisa W, LEE, Wang-Tso, DESHPANDE, Charu, FRECKMANN, Mary-Louise, SHIH, Ling-Yu, WASSERSTEIN, Melissa, RASMUSSEN, Malene B, LUND, Allan M, PROCOPIS, Peter, CAMERON, Jessie M, ROBINSON, Brian H, SWANSON, Michael A, BROWN, Garry K, BROWN, Ruth M, COMPTON, Alison G, DIECKMANN, Carol L, COLLARD, Renata, COUGHLIN, Curtis R, SPECTOR, Elaine, WEMPE, Michael F, VAN HOVE, Johan L. K, SHAIKH, Tamim, BHATTACHARYA, Kaustuv, SCHARER, Gunter H, AICHER, Joseph, CREADON-SWINDELL, Geralyn, GEIGER, Elizabeth, MACLEAN, Kenneth N

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SNARE proteins rescue impaired autophagic flux in Down syndrome von Aivazidis, Stefanos, Jain, Abhilasha, Rauniyar, Abhishek K, Anderson, Colin C, Marentette, John O, Orlicky, David J, Fritz, Kristofer S, Harris, Peter S, Siegel, David, Maclean, Kenneth N, Roede, James R

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Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease von Friederich, Marisa W., Elias, Abdallah F., Kuster, Alice, Laugwitz, Lucia, Larson, Austin A., Landry, Aaron P., Ellwood‐Digel, Logan, Mirsky, David M., Dimmock, David, Haven, Jaclyn, Jiang, Hua, MacLean, Kenneth N., Styren, Katie, Schoof, Jonathan, Goujon, Louise, Lefrancois, Thomas, Friederich, Maike, Coughlin, Curtis R., Banerjee, Ruma, Haack, Tobias B., Van Hove, Johan L. K.

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Dysregulation of hepatic one‐carbon metabolism in classical homocystinuria: Implications of redox‐sensitive DHFR repression and tetrahydrofolate depletion for pathogenesis and trea... von Maclean, Kenneth N., Jiang, Hua, Neill, Philip D., Chanin, Ryan R., Hurt, K. Joseph, Orlicky, David J., Bottiglieri, Teodoro, Roede, James R., Stabler, Sally P.

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Trisomy 21 results in modest impacts on mitochondrial function and central carbon metabolism von Anderson, Colin C., Marentette, John O., Prutton, Kendra M., Rauniyar, Abhishek K., Reisz, Julie A., D'Alessandro, Angelo, Maclean, Kenneth N., Saba, Laura M., Roede, James R.

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Restoration of the ER stress response protein TDAG51 in hepatocytes mitigates NAFLD in mice von Yousof, Tamana R., Bouchard, Celeste C., Alb, Mihnea, Lynn, Edward G., Lhoták, Sárka, Jiang, Hua, MacDonald, Melissa, Li, Hui, Byun, Jae H., Makda, Yumna, Athanasopoulos, Maria, Maclean, Kenneth N., Cherrington, Nathan J., Naqvi, Asghar, Igdoura, Suleiman A., Krepinsky, Joan C., Steinberg, Gregory R., Austin, Richard C.

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Deep postnatal phenotyping of a new mouse model of nonketotic hyperglycinemia von Swanson, Michael A., Jiang, Hua, Busquet, Nicolas, Carlsen, Jessica, Brindley, Connie, Benke, Tim A., Van Hove, Roxanne A., Friederich, Marisa W., MacLean, Kenneth N., Mesches, Michael H., Van Hove, Johan L. K.

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Experimental evidence for therapeutic potential of taurine in the treatment of nonalcoholic fatty liver disease von Gentile, Christopher L, Nivala, Angela M, Gonzales, Jon C, Pfaffenbach, Kyle T, Wang, Dong, Wei, Yuren, Jiang, Hua, Orlicky, David J, Petersen, Dennis R, Pagliassotti, Michael J, Maclean, Kenneth N

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Liver-Specific Deletion of Phosphatase and Tensin Homolog Deleted on Chromosome 10 Significantly Ameliorates Chronic EtOH-Induced Increases in Hepatocellular Damage von Shearn, Colin T, Orlicky, David J, McCullough, Rebecca L, Jiang, Hua, Maclean, Kenneth N, Mercer, Kelly E, Stiles, Bangyan L, Saba, Laura M, Ronis, Martin J, Petersen, Dennis R

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Taurine alleviates repression of betaine‐homocysteine S‐methyltransferase and significantly improves the efficacy of long‐term betaine treatment in a mouse model of cystathionine β... von Maclean, Kenneth N., Jiang, Hua, Phinney, Whitney N., Keating, Amy K., Hurt, K. Joseph, Stabler, Sally P.

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Integrated stress response modulates cellular redox state via induction of cystathionine γ-lyase: cross-talk between integrated stress response and thiol metabolism von Dickhout, Jeffrey G, Carlisle, Rachel E, Jerome, Danielle E, Mohammed-Ali, Zahraa, Jiang, Hua, Yang, Guangdong, Mani, Sarathi, Garg, Sanjay K, Banerjee, Ruma, Kaufman, Randal J, Maclean, Kenneth N, Wang, Rui, Austin, Richard C

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Cystathionine Protects against Endoplasmic Reticulum Stress-induced Lipid Accumulation, Tissue Injury, and Apoptotic Cell Death von Maclean, Kenneth N., Greiner, Lori S., Evans, Jeffrey R., Sood, Sudesh K., Lhotak, Sarka, Markham, Neil E., Stabler, Sally P., Allen, Robert H., Austin, Richard C., Balasubramaniam, Vivek, Jiang, Hua

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Cystathionine γ-lyase promotes estrogen-stimulated uterine artery blood flow via glutathione homeostasis von Bok, Rachael, Guerra, Damian D., Lorca, Ramón A., Wennersten, Sara A., Harris, Peter S., Rauniyar, Abhishek K., Stabler, Sally P., MacLean, Kenneth N., Roede, James R., Brown, Laura D., Hurt, K. Joseph

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Perinatal Choline Supplementation Improves Cognitive Functioning and Emotion Regulation in the Ts65Dn Mouse Model of Down Syndrome von Moon, Jisook, Chen, May, Gandhy, Shruti U, Strawderman, Myla, Levitsky, David A, Maclean, Kenneth N, Strupp, Barbara J

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Altered hepatic sulfur metabolism in cystathionine β‐synthase‐deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways von Jiang, Hua, Stabler, Sally P., Allen, Robert H., Abman, Steven H., Maclean, Kenneth N.

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GDF10 blocks hepatic PPARγ activation to protect against diet-induced liver injury von Platko, Khrystyna, Lebeau, Paul F., Byun, Jae Hyun, Poon, Samantha V., Day, Emily A., MacDonald, Melissa E., Holzapfel, Nicholas, Mejia-Benitez, Aurora, Maclean, Kenneth N., Krepinsky, Joan C., Austin, Richard C.

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Loss of TDAG51 Results in Mature-Onset Obesity, Hepatic Steatosis, and Insulin Resistance by Regulating Lipogenesis von BASSERI, Sana, LHOTAK, Sarka, FULLERTON, Morgan D, PALANIVEL, Rengasamy, HUA JIANG, LYNN, Edward G, FORD, Rebecca J, MACLEAN, Kenneth N, STEINBERG, Gregory R, AUSTIN, Richard C

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Switching obese mothers to a healthy diet improves fetal hypoxemia, hepatic metabolites, and lipotoxicity in non-human primates von Wesolowski, Stephanie R., Mulligan, Christopher M., Janssen, Rachel C., Baker, Peter R., Bergman, Bryan C., D'Alessandro, Angelo, Nemkov, Travis, Maclean, Kenneth N., Jiang, Hua, Dean, Tyler A., Takahashi, Diana L., Kievit, Paul, McCurdy, Carrie E., Aagaard, Kjersti M., Friedman, Jacob E.

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Oxidative stress-mediated aldehyde adduction of GRP78 in a mouse model of alcoholic liver disease: functional independence of ATPase activity and chaperone function von Galligan, James J., Fritz, Kristofer S., Backos, Donald S., Shearn, Colin T., Smathers, Rebecca L., Jiang, Hua, MacLean, Kenneth N., Reigan, Philip R., Petersen, Dennis R.

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