Search Results - "MacLean, Kenneth N."

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

“…Down syndrome (DS) is a genetic disorder caused by trisomy of chromosome 21. Abnormalities in chromosome number have the potential to lead to disruption of the…”
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Variant non-ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5 by 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

“…Patients with nonketotic hyperglycinemia and deficient glycine cleavage enzyme activity, but without mutations in AMT, GLDC or GCSH, the genes encoding its…”
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Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease by 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.

“…Hydrogen sulfide, a signaling molecule formed mainly from cysteine, is catabolized by sulfide:quinone oxidoreductase (gene SQOR). Toxic hydrogen sulfide…”
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SNARE proteins rescue impaired autophagic flux in Down syndrome by 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

“…Down syndrome (DS) is a chromosomal disorder caused by trisomy of chromosome 21 (Ts21). Unbalanced karyotypes can lead to dysfunction of the proteostasis…”
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Experimental evidence for therapeutic potential of taurine in the treatment of nonalcoholic fatty liver disease by 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

“…The incidence of obesity is now at epidemic proportions and has resulted in the emergence of nonalcoholic fatty liver disease (NAFLD) as a common metabolic…”
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Pcsk9 knockout exacerbates diet-induced non-alcoholic steatohepatitis, fibrosis and liver injury in mice by Lebeau, Paul F., Byun, Jae Hyun, Platko, Khrystyna, Al-Hashimi, Ali A., Lhoták, Šárka, MacDonald, Melissa E., Mejia-Benitez, Aurora, Prat, Annik, Igdoura, Suleiman A., Trigatti, Bernardo, Maclean, Kenneth N., Seidah, Nabil G., Austin, Richard C.

“…The fatty acid translocase, also known as CD36, is a well-established scavenger receptor for fatty acid (FA) uptake and is abundantly expressed in many…”
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Evidence for Social Anxiety and Impaired Social Cognition in a Mouse Model of Fragile X Syndrome by McNaughton, Caitlyn H, Moon, Jisook, Strawderman, Myla S, Maclean, Kenneth N, Evans, Jeffrey, Strupp, Barbara J

“…This study assessed social behavior in a mouse model of Fragile X syndrome (FXS), the Fmr1 tm1Cgr or Fmr1 "knockout" (KO) mouse. Both the KO and wild-type (WT)…”
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Methylene-tetrahydrofolate reductase contributes to allergic airway disease by Eyring, Kenneth R, Pedersen, Brent S, Maclean, Kenneth N, Stabler, Sally P, Yang, Ivana V, Schwartz, David A

“…Environmental exposures strongly influence the development and progression of asthma. We have previously demonstrated that mice exposed to a diet enriched with…”
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Cystathionine γ-lyase promotes estrogen-stimulated uterine artery blood flow via glutathione homeostasis by 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

“…During pregnancy, estrogen (E2) stimulates uterine artery blood flow (UBF) by enhancing nitric oxide (NO)-dependent vasodilation. Cystathionine γ-lyase (CSE)…”
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Cystathionine Protects against Endoplasmic Reticulum Stress-induced Lipid Accumulation, Tissue Injury, and Apoptotic Cell Death by 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

“…Cystathionine (R-S-(2-amino-2-carboxyethyl)-l-homocysteine) is a non-proteinogenic thioether containing amino acid. In mammals, cystathionine is formed as an…”
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GDF10 blocks hepatic PPARγ activation to protect against diet-induced liver injury by 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.

“…Growth differentiation factors (GDFs) and bone-morphogenic proteins (BMPs) are members of the transforming growth factor β (TGFβ) superfamily and are known to…”
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Switching obese mothers to a healthy diet improves fetal hypoxemia, hepatic metabolites, and lipotoxicity in non-human primates by 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.

“…Non-alcoholic fatty liver disease (NAFLD) risk begins in utero in offspring of obese mothers. A critical unmet need in this field is to understand the pathways…”
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Loss of TDAG51 Results in Mature-Onset Obesity, Hepatic Steatosis, and Insulin Resistance by Regulating Lipogenesis by 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

“…Regulation of energy metabolism is critical for the prevention of obesity, diabetes, and hepatic steatosis. Here, we report an important role for the…”
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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 by 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

“…Alcoholic liver disease is a significant contributor to global liver failure. In murine models, chronic ethanol consumption dysregulates PTEN/Akt signaling…”
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Oxidative Stress and the ER Stress Response in a Murine Model for Early-Stage Alcoholic Liver Disease by Orlicky, David J., MacLean, Kenneth N., Petersen, Dennis R., Galligan, James J., Franklin, Christopher C., Jiang, Hua, Backos, Donald S., Smathers, Rebecca L., Shearn, Colin T., Fritz, Kristofer S.

“…Alcoholic liver disease (ALD) is a primary cause of morbidity and mortality in the United States and constitutes a significant socioeconomic burden. Previous…”
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Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment by Maclean, Kenneth N., Sikora, Jakub, Kožich, Viktor, Jiang, Hua, Greiner, Lori S., Kraus, Eva, Krijt, Jakub, Crnic, Linda S., Allen, Robert H., Stabler, Sally P., Elleder, Milan, Kraus, Jan P.

“…Cystathionine beta-synthase (CBS) deficient homocystinuria is an inherited metabolic defect that if untreated typically results in mental retardation,…”
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Modulating cognitive deficits and tau accumulation in a mouse model of aging Down syndrome through neonatal implantation of neural progenitor cells by Rachubinski, Angela L., Maclean, Kenneth N., Evans, Jeffrey R., Bjugstad, Kimberly B.

“…Although Down syndrome (DS) is primarily considered as a pediatric disorder, all DS patients incur Alzheimer's disease (AD)-like pathology and about 60%…”
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Effects of neonatal neural progenitor cell implantation on adult neuroanatomy and cognition in the Ts65Dn model of Down syndrome by Rachubinski, Angela L, Crowley, Shannon K, Sladek, Jr, John R, Maclean, Kenneth N, Bjugstad, Kimberly B

“…As much of the aberrant neural development in Down syndrome (DS) occurs postnatally, an early opportunity exists to intervene and influence life-long cognitive…”
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Constitutive induction of pro-inflammatory and chemotactic cytokines in cystathionine beta-synthase deficient homocystinuria by Keating, Amy K., Freehauf, Cynthia, Jiang, Hua, Brodsky, Gary L., Stabler, Sally P., Allen, Robert H., Graham, Douglas K., Thomas, Janet A., Van Hove, Johan L.K., Maclean, Kenneth N.

“…Cystathionine beta-synthase (CBS) deficient homocystinuria (HCU) is an inherited metabolic defect that if untreated, typically results in cognitive impairment,…”
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Deficiency of TDAG51 Protects Against Atherosclerosis by Modulating Apoptosis, Cholesterol Efflux, and Peroxiredoxin‐1 Expression by Hossain, Gazi S., Lynn, Edward G., Maclean, Kenneth N., Zhou, Ji, Dickhout, Jeffrey G., Lhoták, Šárka, Trigatti, Bernardo, Capone, John, Rho, Jaerang, Tang, Damu, McCulloch, Christopher A., Al‐Bondokji, Imtisal, Malloy, Mary J., Pullinger, Clive R., Kane, John P., Li, Yonghong, Shiffman, Dov, Austin, Richard C.

“…Background Apoptosis caused by endoplasmic reticulum (ER) stress contributes to atherothrombosis, the underlying cause of cardiovascular disease (CVD). T‐cell…”
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