Kenneth MacLean, PhD

Publications

• Kenneth N. Maclean, Hua Jiang, Lori S. Greiner, Robert H. Allen, Sally P. Stabler. Long-term betaine therapy in a murine model of cystathionine beta-synthase deficient homocystinuria: Decreased efficacy over time reveals a significant threshold effect between elevated homocysteine and thrombotic risk. Molecular Genetics and Metabolism. 2012 105, 395-403.
• Jeffrey G. Dickhout, Rachel E. Carlisle, Danielle E. Jerome, Zahraa Mohammed-Ali, Hua Jiang, Guangdong Yang, Sarathi Mani, Sanjay Garg, Ruma Banerjee, Randal J. Kaufman, Kenneth N. Maclean, Rui Wang, and Richard C. Austin.. The integrated stress response modulates cellular redox state via induction of cystathionine ?-lyase: cross-talk between the integrated stress response and thiol metabolism. Journal of Biological Chemistry. 2012 Mar 2; 287(10):7603-14.
• Rachubinski A.L., Crowley S.K., Sladek J.R., Maclean K.N., Bjugstad K.B. The effects of neonatal neural progenitor cell implantation on adult neuroanatomy and cognition in the Ts65Dn model of Down syndrome. PLoS one. April 2012. 2012;7(4):e36082. Epub 2012 Apr 25.
• Hua Jiang, Sally P. Stabler Robert H. Allen and Kenneth N. Maclean. Altered expression of apoA-I, apoA-IV and PON-1 activity in CBS deficient homocystinuria in the presence and absence of treatment: Implications for cardiovascular outcomes. Molecular Genetics and Metabolism. 2012 Sep;107(1-2):55-65.
• James J. Galligan, Rebecca L. Smathers, , Colin T. Shearn, Kristofer S. Fritz, Hua Jiang , Donald S. Backos, Christopher C. Franklin, David J. Orlicky, Kenneth N. Maclean, Dennis R. Petersen. Oxidative stress and the ER stress response in a murine model of early-stage alcoholic liver disease. Journal of Toxicology. 2012;2012:207594. Epub 2012 Jul 5.
• Angela L. Rachubinski, Kenneth N. Maclean, Jeffrey R. Evans, Kimberly B. Bjugstad. Modulating cognitive deficits and tau accumulation in a mouse model of aging adult Down syndrome through neonatal implantation of neural progenitor cells Experimental Gerontology. 2012; 47: 723-733. Epub 2012 July7.
• Kenneth N. Maclean, Lori S. Greiner, Jeffrey R. Evans, Sarka Lhotak, Sally P. Stabler, Robert H. Allen, Richard C. Austin, Hua Jiang. Cystathionine protects against endoplasmic reticulum stress induced lipid accumulation and apoptotic cell death. Journal of Biological Chemistry. 2012, 287, (38):31994-32005.
• Kenneth N. Maclean. Betaine treatment of cystathionine beta-synthase deficient homocystinuria; Does it work and can it be improved? Orphan Drugs:Research and Reviews. 2012:2 23-33.
• 38. Sana Basseri, Šárka Lhoták, Morgan D. Fullerton, Rengasamy Palanivel, Hua Jiang, Edward G. Lynn, Rebecca J. Ford, Kenneth N. Maclean, Gregory R. Steinberg, Richard C. Austin. Loss of TDAG51 results in mature-onset obesity, hepatic steatosis, and insulin resistance by regulating adipogenesis and lipogenesis. Diabetes. 2013 Jan;62(1):158-69.
• 39. Colin T. Shearn, Rebecca L. Smathers, David J. Orlicky, Kenneth N. Maclean, and Dennis R. Petersen. Increased dietary fat leads to dysregulation of the LKB1/AMPK pathway and increased damage in an early mouse model of ethanol-mediated steatosis. Journal of Nutritional Biochemistry 2013 Aug;24(8):1436-45.
• 40. Gazi S Hossain, Ji Zhou, Kenneth N. Maclean, Bernardo Trigatti, Stephen Colgan, Sárka Lhoták, Jeffrey G. Dickhout, Ira Tabas, Tracie A. Seimon, Hansa Patel, John Capone, Jaerang Rho, Damu Tang and Richard C Austin. Deficiency of TDAG51 Attenuates Atherogenesis by Negatively Regulating PPAR? Expression. Journal of the American Heart Association. 2013 May 17;2(3):e000134.
• 41. Peter R. Baker II, Marisa W. Friederich, Michael A. Swanson, Tamim Shaikh, Kaustuv Bhattacharya, Gunter H. Scharer, Joseph Aicher, Geralyn Creadon-Swindel, Elizabeth Geiger, Kenneth N. MacLean, Wang-Tso Lee, Charu Deshpande, Mary-Louise Freckmann, Ling-Yu Shih, Melissa Wasserstein, Malene B. Rasmussen, Allan M. Lund, Jesse Cameron, Brian Robinson, Gary K. Brown, Alison G. Compton, Carol L. Dieckmann, Renata Collard, Curtis R. Coughlin II, Elaine Spector, Michael F. Wempe, Johan L.K. Van Hove. Variant non-ketotic hyperglycinemia, a new disorder, is caused by genetic defects in lipoate biosynthesis, including mutations in LIAS, BOLA3, and the novel gene GLRX5. Brain. 2014,137:366-379.
• Maclean K.N., Jiang H., Allen R.H. and Stabler S.P. Taurine Alleviates Oxidative Stress Mediated Repression of Betaine-Homocysteine S-Methyltransferase Resulting in Improved Long-Term Efficacy of Betaine Treatment in a Mouse Model of CBS Deficient Homocystinuria. . 9th Annual International Homocysteine Conference, Dublin, Ireland, 8th-12th September, 2013.
• Sana Basseri, Šárka Lhoták, Morgan D. Fullerton, Rengasamy Palanivel, Hu Jiang, Edward G. Lynn, Rebecca J. Ford, Kenneth N. Maclean, Gregory R. Steinberg, Richard C. Austin. Loss of TDAG51 results in mature-onset obesity, hepatic steatosis, and insulin resistance by regulating adipogenesis and lipogenesis. Diabetes. 2013 Epub 2012 Sep 6.
• Maclean K.N., Jiang H., Galligan J.J., Breen K., Rosen R., Hurt K.J., Allen R.H., Stabler S.P., and Orlicky D.J. Age–Related Hepatopathy in CBS Deficient Homocystinuria: Altered Methionine Cycle Metabolism, Impaired Protein Glycosylation and Methionine Toxicity are ameliorated by Taurine and Betaine Treatment. 9th Annual International Homocysteine Conference, Dublin, Ireland, 8th-12th September, 2013.
• Maclean K.N., Jiang H., Breen K., Allen R. H., Rosen R., Orlicky D.J., Hurt K.J. and Stabler S.P. Gender-Specific Dysregulation of the Methionine Cycle in Cystathionine Gamma-Lyase Null Mice Reveals Possible Hepatoprotective Effects of Elevated Cystathionine. 9th Annual International Homocysteine Conference, Dublin, Ireland. 8th-12th September, 2013.
• . Peter R. Baker II, Marisa W. Friederich, Michael A. Swanson, Tamim Shaikh, Kaustuv Bhattacharya, Gunter H. Scharer, Joseph Aicher, Geralyn Creadon-Swindel, Elizabeth Geiger, Kenneth N. MacLean, Wang-Tso Lee, Charu Deshpande, Mary-Louise Freckmann, Ling-Yu Shih, Melissa Wasserstein, Malene B. Rasmussen, Allan M. Lund, Jesse Cameron, Brian Robinson, Gary K. Brown, Alison G. Compton, Carol L. Dieckmann, Renata Collard, Curtis R. Coughlin II, Elaine Spector, Michael F. Wempe, Johan L.K. Van Hove. Variant non-ketotic hyperglycinemia, a new disorder, is caused by genetic defects in lipoate biosynthesis, including mutations in LIAS, BOLA3, and the novel gene GLRX5. Brain. 2014,137:366-379.
• James J. Galligan, Kristofer S. Fritz, Donald S. Backos, Colin T. Shearn, Rebecca L. Smathers, Hua Jiang, Kenneth N. MacLean, Philip R. Reigan, Dennis R. Petersen. GRP78 Demonstrates Resistance to Electrophile-Mediated Disruption in Chaperone Activity. Free Radical Biology and Medicine. 2014, 73:411-20
• Hua Jiang, Sally P. Stabler, Robert H. Allen, Steven H. Abman, Kenneth N. Maclean Altered hepatic sulfur metabolism in cystathionine beta-synthase deficient homocystinuria: Regulatory role of taurine upon competing cysteine oxidation pathways. FASEB Journal. 2014,28:4044-4054.
• 4. Anita M. Quintana, Elizabeth Geiger, Nate Achilly, David S. Rosenblatt, Kenneth N. Maclean, Sally P. Stabler, Bruce Appel, and Tamim H. Shaik. Hcfc1b, a zebrafish ortholog of HCFC1, regulates craniofacial development by modulating mmachc expression. Developmental Biology. 2014 Dec 1;396(1):94-106
• K. Joseph Hurt, H. Jiang , K. Breen, D.J. Orlicky, S.P. Stabler and K.N. Maclean. Sex-Specific Methionine Cycle Changes in Cystathionine Gamma Lyase Null Mice Suggest Cytoprotection by Cystathionine. Annual meeting of the Society for Gynecological Investigation, March 26-29, 2014.
• Kenneth N. Maclean, Hua Jiang, Robert H. Allen, Sally P. Stabler. Oxidative Stress and epigenetic alteration of gene expression: Towards a unifying theory of pathogenesis in CBS deficient homocystinuria. Plenary session, FASEB Science Research Conference, Folic acid, vitamin B12 and one carbon metabolism August 3-8 2014, Steamboat Springs, Colorado
• Hua Jiang, Kelsey Breen, Robert H. Allen, Sally P. Stabler. David J. Orlicky, K. Joseph Hurt, Kenneth N. Maclean. Sex-specific dysregulation of cysteine oxidation and the methionine and folate cycles in female cystathionine gamma-lyase null mice: A serendipitous model of the methylfolate trap. Biology Open. 2015 Aug. 4,1154-1162
• James D. Weisfeld-Adams, Amanda K. Tkachuk, Kenneth N. Maclean, Naomi L. Meeks, Stuart A. Scott. A de novo 2.78 Mb duplication on chromosome 21q22.11 implicates candidate genes in the partial trisomy 21 phenotype. Nature Partner Journal:Genomic Medicine 2016 March. 1,2056-2059
• Colin T. Shearn, David J. Orlicky, Rebecca L. Smathers-McCullough, Hua Jiang, Kenneth N. Maclean, Kelly E. Mercer, Martin J. J. Ronis, and Dennis R. Petersen Liver-specific deletion of phosphatase and tensin homolog deleted on chromosome 10 prevents induction of oxidative stress by chronic ethanol feeding. PLoS One. 2016 Apr 28;11(4):e0154152.
• Stefanos Aivazidis, Christina M. Coughlan, Abhishek K. Rauniyar, Hua Jiang, L. Alexander Ligget, Kenneth N. Maclean and James R. Roede. The Burden of Trisomy 21 Disrupts the Proteostasis Network in Down Syndrome. PLoS One. In Press
• Rene Jacobs, Hua Jiang, John P. Kenelley, David J. Orlicky, Robert H. Allen, Sally P. Stabler, Kenneth N. Maclean. Cystathionine beta-synthase deficiency alters hepatic phospholipid metabolism: Post-translational repression of phosphatidylethanolamine methyltransferase is a consequence rather than a cause of liver injury in homocystinuria. Molecular Genetics and Metabolism. In Press
• Kenneth R. Eyring, Brent S. Pedersen, Kenneth N. Maclean, Sally P. Stabler, Ivana V. Yang, David A. Schwartz. Allergic Airway Disease is Dependent on Methylene-Tetrahydrofolate Reductase. PloS One. 2018 Jan 12; 13(1): e0190916. .
• Kenneth N. MacLean, Hua Jiang, Stefanos Aivazidis, Eugene Kim, Colin T. Shearn, Peter S. Harris, Dennis R. Petersen, Sally P. Stabler, Robert H. Allen and James R. Roede. Taurine treatment prevents derangement of the hepatic gamma-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: Regulatory cross-talk between thiol and sulfinic acid metabolism. FASEB Journal. 2018 Mar; 32(3):1265-1280.
• Stephanie R Wesolowski, Christopher M Mulligan, Bryan C Bergman, Kenneth N. Maclean, Hua Jiang, Rachel C Janssen, Angelo D’Alessandro, Travis Nemkov, Tyler Dean, Diana Takahashi, Carrie Paul Kievit, E. McCurdy, Kevin L Grove, Kjersti M. Aagaard, and Jacob E Friedman. Switching Obese Mothers to a Healthy Diet Improves Hypoxia and Partially Restores Fetal Liver and Serum Metabolome in Non-Human Primates. Molecular Metabolism.2018 Dec; 18:25-41.
• Kenneth N. Maclean, Hua Jiang, Whitney N. Phinney, Amy K. Keating, K. Joseph Hurt, Sally P. Stabler. Taurine alleviates repression of betaine-homocysteine S-methyltransferase and significantly improves the efficacy of long-term betaine treatment in a mouse model of cystathionine ß-synthase deficient homocystinuria. In Press FASEB Journal.
• Michael A. Swanson, Leilani Rodriguez, Hua Jiang, Kaz Knight, Marisa Friederich, Nicholas Greene, Linda Johnson, Kenneth MacLean, and Johan Van Hove. characterization of a new nonketotic hyperglycinemia mouse model with an unstable missense mutation in GLDC . SIMD, San Diego, March 2018.
• Kenneth N. Maclean, Hua Jiang, David J. Orlicky, Eugene Kim, Sally P. Stabler. Classical homocystinuria induces hypersensitivity to acetaminophen toxicity and liver failure by severely impairing drug sulfation and glucuronidation, thiol depletion and constitutive induction of Cyp2e1: Implications for therapy. FASEB Science Research Conference, Folic acid, vitamin B12 and one carbon metabolism July 29-August 4 2018, Halifax, Nova Scotia, Canada.
• Marisa W. Friederich, Austin A. Larson, Aaron Landry, David Dimmock, Hua Jiang, Kenneth MacLean, Ruma Bannerjee, Johan L.K. Van Hove. Mutations in SQOR encoding sulfide:quinone oxidoreductase causes fatal Leigh disease. The 6th biennial Australian conference for mitochondrial research, 29 November 29 –1 December 1st, Melbourne, Australia.
• Rene Jacobs, Hua Jiang, John P. Kenelley, David J. Orlicky, Robert H. Allen, Sally P. Stabler, Kenneth N. Maclean. Cystathionine beta-synthase deficiency alters hepatic phospholipid metabolism: Post-translational repression of phosphatidylethanolamine methyltransferase is a consequence rather than a cause of liver injury in homocystinuria. Molecular Genetics and Metabolism. 2017. 120, 325–336
• Stefanos Aivazidis, Christina M. Coughlan, Abhishek K. Rauniyar, Hua Jiang, L. Alexander Ligget, Kenneth N. Maclean and James R. Roede. The Burden of Trisomy 21 Disrupts the Proteostasis Network in Down Syndrome. PLoS One. 2017 Apr;120(4):325-336.
• Kenneth N. MacLean, Hua Jiang, Stefanos Aivazidis, Eugene Kim, Colin T. Shearn, Peter S. Harris, Dennis R. Petersen, Sally P. Stabler, Robert H. Allen and James R. Roede. Taurine treatment prevents derangement of the hepatic gamma-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: Regulatory cross-talk between thiol and sulfinic acid metabolism. FASEB Journal. In Press.
• 1. Kenneth N. Maclean, Hua Jiang, Whitney N. Phinney, Amy K. Keating, K. Joseph Hurt, Sally P. Stabler. Taurine alleviates repression of betaine-homocysteine S-methyltransferase and significantly improves the efficacy of long-term betaine treatment in a mouse model of cystathionine ß-synthase deficient homocystinuria. FASEB Journal. 2019 May;33(5):6339-6353
• 2. Johan L.K. Van Hove, Cynthia L. Freehauf, Can Ficicioglu, Loren Del Mar Pena, Kerrie L. Moreau, Thomas K. Henthorn, Uwe Christians, Hua Jiang, Tina Cowan, Sarah Young, Michelle Hite, Marisa W. Friederich, Sally Stabler, Elaine Spector, Kathryn Kronquist, Janet A. Thomas, Peggy Emmett, Mary J. Harrington, Laura Pyle, Michael Wempe, Kenneth N. MacLean. Biomarkers of Oxidative Stress, Inflammation, and Vascular Dysfunction in Inherited Cystathionine ß-synthase Deficient Homocystinuria and the Impact of Taurine Treatment in a Phase1/2 human clinical trial. Journal of Inherited Metabolic Disease, 2019 May;42(3):424-437.
• 3. Khrystyna Platko, Paul Lebeau, Jae Hyun Byun, Samantha Poon, Melissa McDonald, Kenneth N. Maclean, Joan C. Krepinsky, Aurora Mejia-Benitez and Richard C. Austin.GDF10 blocks hepatic PPAR? activation to protect against diet-induced NASH in mice. Molecular Metabolism 27(2019) 62-74.
• 4. Stefanos Aivazidis, Abhilasha Jain, Colin C. Anderson, David J. Orlicky, Abhishek K. Rauniyar, Kristofer S. Fritz, Peter S. Harris, David Siegel, Kenneth N. Maclean and James R. Roede. Reconstitued expression of SNARE proteins rescues impaired autophagy in Down syndrome. In Press at PloS ONE.
• Kenneth R. Eyring, Brent S. Pedersen, Kenneth N. Maclean, Sally P. Stabler, Ivana V. Yang, David A. Schwartz. Allergic Airway Disease is Dependent on Methylene-Tetrahydrofolate Reductase. PloS One. In Press.
• 5. Paul Lebeau, Jae Hyun Byun, Khrystyna Platko, Ali A. Al-Hashimi, Šárka Lhoták , Samantha Poon, Aurora Mejia-Benitez, Annik Prat, Suleiman A. Igdoura, Bernardo Trigatti, Kenneth N. Maclean, Nabil G. Seidah, Richard C. Austin. Circulating PCSK9 prevents diet-induced hepatic steatosis and attenuates ER stress and liver injury in mice. In Press at Journal of Hepatology Reports.
• Kenneth N. Maclean, Hua Jiang, David J. Orlicky, Eugene Kim, Sally P. Stabler. Classical homocystinuria induces hypersensitivity to acetaminophen toxicity and liver failure by severely impairing drug sulfation and glucuronidation, thiol depletion and constitutive induction of Cyp2e1: Implications for therapy. 12th International Conference on One Carbon Metabolism, B Vitamins and Homocysteine. June 9th to 13th 2019, Tarragona, Spain.
• Kenneth N. Maclean, Hua Jiang, Johan L.K. Van Hove, Sally P. Stabler. Manipulating folate metabolism to achieve near-normal homocysteine levels in a mouse model of homocystinuria due to CBS deficiency: Is this the end of dietary methionine restriction? SSIEM International Meeting. Rotterdam, Netherlands. 4th September 2019.
• Van Hove, JLK, Friederich M.W., Elias A.F., Larson A., Landry A., Ellwood-Digel L., Mirsky D., Dimmock D., Haven J., Friederich M., Jiang H., Maclean K.N., Banerjee R. Mutations in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease. SSIEM International Meeting. Rotterdam, Netherlands. 5th September 2019.
• Marisa W. Friederich, Austin A. Larson, Aaron Landry, David Dimmock, Hua Jiang, Kenneth N. MacLean, Ruma Bannerjee, Johan L.K. Van Hove. Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease. In Press at Journal of Inherited Metabolic Disease. 2020 Sep;43(5):1024-1036. PMID: 32160317
• Anne C Porter, Diane L Gumina, Rachael Bok, Kenneth N Maclean, Nichole Reisdorph, Henry L Galan, Sally P Stablem+r, Beth A Bailey, John C Hobbins, K. Joseph Hurt. Maternal amino acid profiles to distinguish pathologic versus constitutional fetal growth restriction defined by Doppler ultrasound: a pilot study. American Journal of Perinatology 2020 Sep;37(11):1084-1093.
• Damian D. Guerra, Rachael Bok, Kelsey Breen, Vibhuti Vyas, Hua Jiang, Kenneth N. MacLean, and K. Joseph Hurt. Estrogen regulates local cysteine metabolism in mouse myometrium. Reproductive Sciences 2021 Jan;28(1):79-90.
• Rachael Bok, Abhishek K. Rauniyar, Peter S. Harris, Sara A. Wennersten, Sally P. Stabler, Laura D. Brown, James R. Roede, Kenneth N. MacLean and K. Joseph Hurt. Redox Regulation of Estrogen-Stimulated Uterine Blood Flow: A Mechanism of Fetal Growth Restriction. In Press at Redox Biology.
• Kenneth N. Maclean, Hua Jiang, Whitney N. Phinney, Bailey M. Mclagan, James R. Roede, Sally P. Stabler. Metabolic derangement of hepatic polyamine, folate and methionine cycle metabolism in cystathionine ß-Synthase-Deficient Homocystinuria in the Presence and Absence of Treatment: Possible Implications for Pathogenesis. In Press at Molecular Genetics and Metabolism.
• Rachael Bok, Abhishek K. Rauniyar, Peter S. Harris, Sara A. Wennersten, Sally P. Stabler, Laura D. Brown, James R. Roede, Kenneth N. MacLean and K. Joseph Hurt. Redox Regulation of Estrogen-Stimulated Uterine Blood Flow: A Mechanism of Fetal Growth Restriction. Annual Meeting of the Society for Reproductive Investigation. Vancouver, Canada. 10-14 Mar 2020.
• 2. Kendra M. Prutton, John O. Marentette, Brice A. Leifheit, Hector Esquer, Daniel V. LaBarbera, Colin C. Anderson, Kenneth N. Maclean and James R. Roede Oxidative stress as a candidate mechanism for accelerated neuroectodermal differentiation due to Trisomy 21 In Press at Free Radical Biology and Medicine 2022 Jun;186:32-42.
• Celeste Bouchard, Tamana R. Yousof, Mihnea Alb, Edward G. Lynn, Sárka Lhoták, Kenneth N. Maclean, Hui Li, Nathan Cherrington, Joan C. Krepinsky, Alistair J. Ingram, Gregory R. Steinberg, Richard C. Austin. Restoration of hepatic TDAG51 expression improves insulin signaling and reduces weight gain in mice. In Press at. J. Biol. Chem
• Kendra M. Prutton, John O. Marentette, Kenneth N. Maclean, James R. Roede. Mitochondrial dynamics in 2-D neural differentiation of Down syndrome induced pluripotent stem cells. In Press at Free Radical Biology and Medicine
• Kristofer Fritz, Hua Jiang, David Orlicky, Rima Rosen, Alana K. Majors, Sally P Stabler James R Roede, Kenneth N Maclean. Protein carbonylation of cysteine residues in classical Homocystinuria:A mechanism to explain the HCU specific Marfanoid Habitus and ectopia lentis. 4th International conference on Rare Diseases, Vienna, December 7-8th 2022
• 1. Damian D. Guerra, Rachael Bok, Kelsey Breen, Vibhuti Vyas, Hua Jiang, Kenneth N. MacLean, and K. Joseph Hurt. Estrogen regulates local cysteine metabolism in mouse myometrium. Reproductive Sciences 2021 Jan;28(1):79-90.
• Kendra M. Prutton, John O. Marentette, Kenneth N. Maclean, James R. Roede. Characterization of mitochondrial and metabolic alterations induced by trisomy 21 during neural differentiation Free Radical Biology and Medicine 196 (2023) 11-21
• Tamana R. Yousof, Celeste C. Bouchard, Mihnea Alb, Edward G. Lynn, Sárka Lhoták, Hua Jiang, Melissa MacDonald, Jae H. Byun, Yumna Makda, Maria Athanasopoulos, Hui Li, Kenneth N. Maclean, Nathan Cherrington, Asghar Naqvi, Suleiman Igdoura, Joan C. Krepinsky, Gregory R. Steinberg, and Richard C. Austin. The restoration of TDAG551 in hepatocytes reduces obesity in mice with NAFLD. In Press at J. Biol. Chem..
• Kenneth N. Maclean, Hua Jiang, Philip D. Neill, Ryan R. Chanin, K. Joseph Hurt, David J. Orlicky, Teodoro Bottiglieri, James R. Roede, Sally P. Stabler. Dysregulation of hepatic one-carbon metabolism in classical homocystinuria: Implications of redox-sensitive DHFR repression and THF depletion for pathogenesis and treatment. In Press at FASEB J.
• Kenneth N Maclean, Hua Jiang, Jan Pícha and Jirí Jirácek, Sally P. Stabler. Characterization of aberrant hepatic and hippocampal one-carbon metabolism in CBS deficient homocystinuria yields new insights into pathogenesis and dramatically improved treatment: The end of methionine restriction? HCY2023/CluB-12 conference. Old divinity school, St John’s college, University of Cambridge, September 17th -20th,2023
• 2. Rachael Bok, Abhishek K. Rauniyar, Peter S. Harris, Sara A. Wennersten, Sally P. Stabler, Laura D. Brown, James R. Roede, Kenneth N. MacLean and K. Joseph Hurt. Redox Regulation of Estrogen-Stimulated Uterine Blood Flow: A Mechanism of Fetal Growth Restriction. Redox Biology. 2021 Apr;40:101827
• 3. Kenneth N. Maclean, Hua Jiang, Whitney N. Phinney, Bailey M. Mclagan, James R. Roede, Sally P. Stabler. Metabolic derangement of hepatic polyamine, folate and methionine cycle metabolism in cystathionine ß-Synthase-Deficient Homocystinuria in the Presence and Absence of Treatment: Possible Implications for Pathogenesis. Molecular Genetics and Metabolism 2021 Feb;132(2):128-138.
• 4. S. Gupta. L. Wang, M. Slifker ,K. Qi, K.N. Maclean., T. Bottiglieri, W.D.Kruger. Analysis of neonatal lethality in CBS deficient mice using metabolic profiling. FASEB J. 2021 Jun;35(6):e21629.
• 5. Anderson C.C., Marentette J.O., Prutton K.M., Rauniyar A.K., Reisz J.A., D’Alessandro A., Maclean K.N., Roede J.R. Trisomy 21 results in modest impacts on mitochondrial function and central carbon metabolism. Free Radical Biology and Medicine 172 (2021) 201-212.
• 6. Colin C. Anderson, Abhishek K. Rauniyar, Abhilasha Jain, Stefanos Aivazidis, John O. Marentette, Kenneth N. Maclean and James R. Roede. The impact of trisomy 21 on the peroxiredoxin-thioredoxin-thioredoxin reductase thiol redox system In Press at Antioxidants
• 7. Celeste Bouchard, Tamana R. Yousof, Mihnea Alb, Edward G. Lynn, Sárka Lhoták, Kenneth N. Maclean, Hui Li, Nathan Cherrington, Joan C. Krepinsky, Alistair J. Ingram, Gregory R. Steinberg, Richard C. Austin. Restoration of hepatic TDAG51 expression improves insulin signaling and reduces weight gain in mice. In Press at. J. Biol. Chem
• 8. Kendra M. Prutton, John O. Marentette, Brice A. Leifheit, Hector Esquer, Daniel V. LaBarbera, Colin C. Anderson, Kenneth N. Maclean and James R. Roede Oxidative stress as a candidate mechanism for accelerated neuroectodermal differentiation due to Trisomy 21 In Press at Stem Cell Reports
• Maclean KN, Jiang H, Stabler SP. Can a better understanding of the impact of HCU upon one-carbon metabolism lead to improved betaine treatment and the end of methionine restriction? 13th International Conference on One Carbon Metabolism, B Vitamins and Homocysteine. September 9th to 13th 20201, Poznan, Poland.
• Maclean K.N. How elucidating regulatory cross-talk between folate and thiol metabolism can lead to improved treatment in classical homocystinuria: A possible route to the end of methionine restriction? NIH Webinar on Organic Acidemias and Homocystinurias - June 24-25th, 2021
• Maclean K.N. Oxidative Stress and epigenetic alteration of gene expression: Towards a unifying theory of pathogenesis in CBS deficient homocystinuria. Travere Advisory Board conference on the Pathophysiology of Homocysteine. August 2021
• Navigating the effects of altered drug metabolism in classical homocystinuria. The 4th International Homocystinurias Patient - Expert Meeting, Melbourne, Australia. 30th November 2021

Practice Locations

Children's Hospital Colorado Anschutz Medical Campus
13123 East 16th Ave
Aurora, CO 80045
720-777-1234

Hospital Affiliation

• Children's Hospital Colorado
• University of Colorado Hospital

Specialty Information

Specialties

• Clinical Genetics and Genomics (MD)

Conditions & Treatments

• Genetic Conditions and Testing
• Food, Nutrition and Metabolism