Nichols Lab

Publications

Harvey, LD; Alotaibi, M; Tai, Y; Tang, Y; Kim, HJ; Kelly, NJ; Sun, W; Woodcock, CC; Arshad, S; Culley, MK; Bertero, T; Benza, RL; Jain, M; Chan, SY. Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension. Science. 2025; 387:eadn7277.

Griffiths, M; Simpson, CE; Yang, J; Vaidya, D; Nies, MK; Brandal, S; Damico, R; Hassoun, P; Ivy, DD; Austin, ED; Benza, RL; Nichols, WC; Manlhiot, C; Everett, AD. Equivalency of Multiple Biomarkers to Clinical Pulmonary Arterial Hypertension Survival Risk Models. Chest. 2024; 166:1511-1531.

Petrow, E; Feng, C; Frazer-Abel, A; Marangoni, RG; Lutz, K; Nichols, WC; Holers, VM; Ritchlin, C; White, RJ; Korman, BD. Utility of factor D and other alternative complement factors as biomarkers in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH). Seminars in Arthritis and Rheumatism. 2024; 69:152554.

Gao, L; Skinner, J; Nath, T; Lin, Q; Griffiths, M; Damico, RL; Pauciulo, MW; Nichols, WC; Hassoun, PM; Everett, AD; Johns, RA. Resistin predicts disease severity and survival in patients with pulmonary arterial hypertension. Respiratory Research. 2024; 25:235.

Emon, I; Al-Qazazi, R; Rauh, M; Vlasschaert, C; Ott, B; McNaughton, A; Potus, F; Pauciulo, M; Bick, A; Nichols, W; Chung, W; Archer, S. Abstract 4138731: Clonal Hematopoiesis of Indeterminant Potential is Associated with Pulmonary Arterial Hypertension. Circulation. 2024; 150:a4138731.

Byrd, C; Benza, R; Webb, A; Schramm, J; Yang, J; Griffiths, M; Brandal, S; Damico, R; Vaidya, D; Simpson, C; Hassoun, P; Everett, A; Austin, E; Martin, L. Abstract 4138019: eQTL Analysis of the PAH Biomarker CCN2 Identifies a Novel SNP that Associates with Survival. Circulation. 2024; 150:a4138019.

Hindmarch, C; Potus, F; Al-Qazazi, R; Nichols, W; Pauciulo, M; Rauh, M; Archer, S. Abstract 4140468: Tet Methylcytosine Dioxygenase 2 mutation drives a global hypermethylation signature in patients with pulmonary arterial hypertension resulting in a common T cell phenotype. Circulation. 2024; 150:a4140468.

Hong, J; Medzikovic, L; Sun, W; Wong, B; Ruffenach, G; Rhodes, CJ; Brownstein, A; Liang, LL; Aryan, L; Li, M; Geraci, MW; Stearman, RS; Yang, X; Eghbali, M. Integrative Multiomics in the Lung Reveals a Protective Role of Asporin in Pulmonary Arterial Hypertension. Circulation. 2024; 150:1268-1287.

Austin, ED; Aldred, MA; Alotaibi, M; Gräf, S; Nichols, WC; Trembath, RC; Chung, WK. Genetics and precision genomics approaches to pulmonary hypertension. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2024; 64:2401370.

Tang, H; Gupta, A; Morrisroe, SA; Bao, C; Schwantes-An, TH; Gupta, G; Liang, S; Sun, Y; Chu, A; Luo, A; Duarte, JD; Nichols, WC; Garcia, JG N; Desai, AA. Deficiency of the Deubiquitinase UCHL1 Attenuates Pulmonary Arterial Hypertension. Circulation. 2024; 150:302-316.

Tai, YY; Yu, Q; Tang, Y; Sun, W; Kelly, NJ; Okawa, S; Zhao, J; Schwantes-An, TH; Lacoux, C; Torrino, S; Nichols, WC; Desai, AA; Bertero, T; Chan, SY. Allele-specific control of rodent and human lncRNA KMT2E-AS1 promotes hypoxic endothelial pathology in pulmonary hypertension. Science Translational Medicine. 2024; 16:eadd2029.

Jose, A; Elwing, JM; Kawut, SM; Pauciulo, MW; Sherman, KE; Nichols, WC; Fallon, MB; McCormack, FX. Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension. Communications Biology. 2023; 6:826.

Welch, CL; Aldred, MA; Balachandar, S; Dooijes, D; Eichstaedt, CA; Gräf, S; Houweling, AC; Machado, RD; Pandya, D; Prapa, M; Noordegraaf, AV; Wilkins, MR; Zamanian, RT; Zateyshchikov, D. Defining the clinical validity of genes reported to cause pulmonary arterial hypertension. Genetics in Medicine. 2023; 25:100925.

Alotaibi, M; Liu, Y; Magalang, GA; Kwan, AC; Ebinger, JE; Nichols, WC; Pauciulo, MW; Jain, M; Cheng, S. Deriving Convergent and Divergent Metabolomic Correlates of Pulmonary Arterial Hypertension. Metabolites. 2023; 13:802.

Torres, G; Lancaster, AC; Yang, J; Griffiths, M; Brandal, S; Damico, R; Vaidya, D; Simpson, CE; Martin, LJ; Pauciulo, MW; Nichols, WC; Ivy, DD; Austin, ED; Hassoun, PM; Everett, AD. Low-affinity insulin-like growth factor binding protein 7 and its association with pulmonary arterial hypertension severity and survival. Pulmonary Circulation. 2023; 13:e12284.

Ortega, R; Burbulla, L; Mileva, I; Taylor, G; Cuperlovic-Culf, M; Glickman, A; Pullman, M; Scherzer, C; Raymond, D; Miltenyi-Miltenberger, G; Obeid, L; Hannun, Y; Bennett, S; Saunders-Pullman, R. Peripheral Sphingolipids as Potential Biomarkers of Parkinson disease Including Sex-Related Differences (P3-11.007). Neurology. 2023; 100.

Sangam, S; Sun, X; Schwantes-An, TH; Yegambaram, M; Lu, Q; Shi, Y; Cook, T; Fisher, A; Frump, AL; Coleman, A; Tang, H; Nichols, WC; Black, SM; Desai, AA. SOX17 Deficiency Mediates Pulmonary Hypertension: At the Crossroads of Sex, Metabolism, and Genetics. American Journal of Respiratory and Critical Care Medicine. 2023; 207:1055-1069.

Torres, G; Yang, J; Griffiths, M; Brandal, S; Damico, R; Vaidya, D; Simpson, CE; Pauciulo, MW; Nichols, WC; Ivy, DD; Austin, ED; Hassoun, PM; Everett, AD. Insulin-like growth factor binding Protein-4: A novel indicator of pulmonary arterial hypertension severity and survival. Pulmonary Circulation. 2023; 13:e12235.

Prohaska, CC; Zhang, X; Schwantes-An, TH L; Stearman, RS; Hooker, S; Kittles, RA; Aldred, MA; Lutz, KA; Pauciulo, MW; Nichols, WC; Desai, AA; Gordeuk, VR; Machado, RF. RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension. Pulmonary Circulation. 2023; 13:e12227.

Eichstaedt, CA; Belge, C; Chung, WK; Gräf, S; Grünig, E; Montani, D; Quarck, R; Tenorio-Castano, JA; Soubrier, F; Trembath, RC; Welch, C; Wilkins, MR; Zamanian, RT; Zateyshchikov, D. Genetic counselling and testing in pulmonary arterial hypertension: a consensus statement on behalf of the International Consortium for Genetic Studies in PAH. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2023; 61:2201471.

Alotaibi, M; Shao, J; Pauciulo, MW; Nichols, WC; Hemnes, AR; Malhotra, A; Kim, NH; Yuan, JX J; Fernandes, T; Kerr, KM; Long, T; Cheng, S; Chan, SY; Jain, M. Metabolomic Profiles Differentiate Scleroderma-PAH From Idiopathic PAH and Correspond With Worsened Functional Capacity. Chest. 2023; 163:204-215.

Harbaum, L; Rhodes, CJ; Wharton, J; Lawrie, A; Karnes, JH; Desai, AA; Nichols, WC; Humbert, M; Montani, D; Girerd, B; Graf, S; Pietzner, M; Morrell, NW; Wilkins, MR. Mining the Plasma Proteome for Insights into the Molecular Pathology of Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. 2022; 205:1449-1460.

Simpson, CE; Griffiths, M; Yang, J; Nies, MK; Vaidya, D; Brandal, S; Martin, LJ; Pauciulo, MW; Lutz, KA; Coleman, AW; Nichols, WC; Everett, AD; Hassoun, PM; Damico, RL. COL18A1 genotypic associations with endostatin levels and clinical features in pulmonary arterial hypertension: a quantitative trait association study. ERJ Open Research. 2022; 8:725-2021.

Toshner, M; Church, C; Harbaum, L; Rhodes, C; Villar Moreschi, SS; Liley, J; Jones, R; Arora, A; Batai, K; Desai, AA; Wilkins, MR; Gaine, S; Morrell, NW; Corris, PA. Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2022; 59:2002463.

Pal, G; Mangone, G; Hill, EJ; Ouyang, B; Liu, Y; Lythe, V; Ehrlich, D; Saunders-Pullman, R; Shanker, V; Bressman, S; Foltynie, T; Hall, D; Corvol, JC; Goetz, CG. Parkinson Disease and Subthalamic Nucleus Deep Brain Stimulation: Cognitive Effects in GBA Mutation Carriers. Annals of Neurology. 2022; 91:424-435.

Griffiths, M; Yang, J; Vaidya, D; Nies, M; Brandal, S; Ivy, DD; Hickey, F; Wolter-Warmerdam, K; Austin, ED; Mullen, M; Hirsch, R; Yung, D; Nichols, WC; Everett, AD. Biomarkers of Pulmonary Hypertension Are Altered in Children with Down Syndrome and Pulmonary Hypertension. The Journal of Pediatrics. 2022; 241:68-76.e3.

Yang, J; Ambade, AS; Nies, M; Griffiths, M; Damico, R; Vaidya, D; Brandal, S; Pauciulo, MW; Lutz, KA; Coleman, AW; Nichols, WC; Austin, ED; Ivy, D; Hassoun, PM; Everett, AD. Hepatoma-derived growth factor is associated with pulmonary vascular remodeling and PAH disease severity and survival. Pulmonary Circulation. 2022; 12:e12007.

Zhu, N; Swietlik, EM; Welch, CL; Pauciulo, MW; Hagen, JJ; Zhou, X; Guo, Y; Karten, J; Pandya, D; Tilly, T; Wharton, J; Wild, J; Wort, SJ; Church, C. Rare variant analysis of 4241 pulmonary arterial hypertension cases from an international consortium implicates FBLN2, PDGFD, and rare de novo variants in PAH. Genome Medicine: medicine in the post-genomic era. 2021; 13:80.

Daly, CM; Griffiths, M; Simpson, CE; Yang, J; Damico, RL; Vaidya, RD; Williams, M; Brandal, S; Jone, PN; Polsen, C; Rosenzweig, EB; Hirsch, R; Yung, D; Everett, AD. Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension. Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease. 2021; 10:e021409.

Simpson, CE; Griffiths, M; Yang, J; Nies, MK; Vaidya, RD; Brandal, S; Martin, LJ; Pauciulo, MW; Lutz, KA; Coleman, AW; Nichols, WC; Everett, AD; Hassoun, PM; Damico, RL. The angiostatic peptide endostatin enhances mortality risk prediction in pulmonary arterial hypertension. ERJ Open Research. 2021; 7:378-2021.

David, FJ; Munoz, MJ; Shils, JL; Pauciulo, MW; Hale, PT; Nichols, WC; Afshari, M; Sani, S; Verhagen Metman, L; Corcos, DM; Pal, GD. Subthalamic Peak Beta Ratio Is Asymmetric in Glucocerebrosidase Mutation Carriers With Parkinson's Disease: A Pilot Study. Frontiers in Neurology. 2021; 12:723476.

Griffiths, M; Yang, J; Nies, M; Damico, RL; Ivy, DD; Martin, W; Nichols, B; Pauciulo, M; Everett, AD. (2021) ST2 Is a Biomarker of Pediatric Pulmonary Arterial Hypertension Severity and Clinical Worsening. Elsevier BV. 160:297-306.

Oldham, WM; Hemnes, AR; Aldred, MA; Barnard, J; Brittain, EL; Chan, SY; Cheng, F; Cho, MH; Desai, AA; Garcia, JG N; Fessel, JP; Aggarwal, NR; Loscalzo, J; Xiao, L. NHLBI-CMREF Workshop Report on Pulmonary Vascular Disease Classification: JACC State-of-the-Art Review. Journal of the American College of Cardiology. 2021; 77:2040-2052.

Moran, EE; Bressman, SB; Ortega, RA; Raymond, D; Nichols, WC; Palmese, CA; Elango, S; Swan, M; Shanker, V; Perera, I; Wang, C; Zimmerman, ME; Saunders-Pullman, R. Cognitive Functioning of Glucocerebrosidase (GBA) Non-manifesting Carriers. Frontiers in Neurology. 2021; 12:635958.

Swietlik, EM; Greene, D; Zhu, N; Megy, K; Cogliano, M; Rajaram, S; Pandya, D; Tilly, T; Lutz, KA; Welch, CC L; Swift, AJ; Nichols, WC; Morrell, NW; Gräf, S. Bayesian Inference Associates Rare KDR Variants with Specific Phenotypes in Pulmonary Arterial Hypertension. Circulation: Genomic and Precision Medicine. 2021; 14:e003155.

Badlam, JB; Badesch, DB; Austin, ED; Benza, RL; Chung, WK; Farber, HW; Feldkircher, K; Frost, AE; Poms, AD; Lutz, KA; Simon, M; White, RJ; Robbins, IM; Chakinala, MM. United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest. 2021; 159:311-327.