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Study of Environmental Enteropathy and Malnutrition (SEEM) in Pakistan: protocols for biopsy based biomarker discovery and validation.

Iqbal NT, Syed S, Sadiq K, Khan MN, Iqbal J, Ma JZ, Umrani F, Ahmed S, Maier EA, Denson LA, Haberman Y, McNeal MM, Setchell KDR, Zhao X, Qureshi S, Shen L, Moskaluk CA, Liu TC, Yilmaz O, Brown DE, Barratt MJ, Kung VL, Gordon JI, Moore SR, Ali SA.

BMC Pediatr. 2019 Jul 22;19(1):247. doi: 10.1186/s12887-019-1564-x.


The FOXM1 Inhibitor RCM-1 Decreases Carcinogenesis and Nuclear β-Catenin.

Shukla S, Milewski D, Pradhan A, Rama N, Rice K, Le T, Flick MJ, Vaz S, Zhao X, Setchell KD, Logarinho E, Kalinichenko VV, Kalin TV.

Mol Cancer Ther. 2019 Jul;18(7):1217-1229. doi: 10.1158/1535-7163.MCT-18-0709. Epub 2019 Apr 30.


Combination of acid β-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype.

Liou B, Zhang W, Fannin V, Quinn B, Ran H, Xu K, Setchell KDR, Witte D, Grabowski GA, Sun Y.

Sci Rep. 2019 Apr 3;9(1):5571. doi: 10.1038/s41598-019-41914-7.


Δ4-3-oxosteroid-5β-reductase deficiency: Responses to oral bile acid therapy and long-term outcomes.

Zhang MH, Setchell KD, Zhao J, Gong JY, Lu Y, Wang JS.

World J Gastroenterol. 2019 Feb 21;25(7):859-869. doi: 10.3748/wjg.v25.i7.859.


Bile Acid Synthesis Disorder Masquerading as Intractable Vitamin D-Deficiency Rickets.

Ahmad O, Nogueira J, Heubi JE, Setchell KDR, Ashraf AP.

J Endocr Soc. 2018 Dec 31;3(2):397-402. doi: 10.1210/js.2018-00314. eCollection 2019 Feb 1.


l-Arginine Synthesis from l-Citrulline in Myeloid Cells Drives Host Defense against Mycobacteria In Vivo.

Lange SM, McKell MC, Schmidt SM, Zhao J, Crowther RR, Green LC, Bricker RL, Arnett E, Köhler SE, Schlesinger LS, Setchell KDR, Qualls JE.

J Immunol. 2019 Mar 15;202(6):1747-1754. doi: 10.4049/jimmunol.1801569. Epub 2019 Feb 1.


Progranulin associates with hexosaminidase A and ameliorates GM2 ganglioside accumulation and lysosomal storage in Tay-Sachs disease.

Chen Y, Jian J, Hettinghouse A, Zhao X, Setchell KDR, Sun Y, Liu CJ.

J Mol Med (Berl). 2018 Dec;96(12):1359-1373. doi: 10.1007/s00109-018-1703-0. Epub 2018 Oct 20.


Placebo-Controlled Randomized Trial of an Intestinal Bile Salt Transport Inhibitor for Pruritus in Alagille Syndrome.

Shneider BL, Spino C, Kamath BM, Magee JC, Bass LM, Setchell KD, Miethke A, Molleston JP, Mack CL, Squires RH, Murray KF, Loomes KM, Rosenthal P, Karpen SJ, Leung DH, Guthery SL, Thomas D, Sherker AH, Sokol RJ; Childhood Liver Disease Research Network.

Hepatol Commun. 2018 Sep 24;2(10):1184-1198. doi: 10.1002/hep4.1244. eCollection 2018 Oct.


Inborn Errors of Bile Acid Metabolism.

Heubi JE, Setchell KDR, Bove KE.

Clin Liver Dis. 2018 Nov;22(4):671-687. doi: 10.1016/j.cld.2018.06.006. Epub 2018 Aug 22. Review.


Infant cholestasis patient with a novel missense mutation in the AKR1D1 gene successfully treated by early adequate supplementation with chenodeoxycholic acid: A case report and review of the literature.

Wang HH, Wen FQ, Dai DL, Wang JS, Zhao J, Setchell KD, Shi LN, Zhou SM, Liu SX, Yang QH.

World J Gastroenterol. 2018 Sep 21;24(35):4086-4092. doi: 10.3748/wjg.v24.i35.4086. Review.


Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders.

Heubi JE, Setchell KDR, Bove KE.

Case Rep Gastroenterol. 2018 Jun 28;12(2):360-372. doi: 10.1159/000490095. eCollection 2018 May-Aug.


Serum Unconjugated Bile Acids and Small Bowel Bacterial Overgrowth in Pediatric Intestinal Failure: A Pilot Study.

Galloway D, Mezoff E, Zhang W, Byrd M, Cole C, Aban I, Kocoshis S, Setchell KD, Heubi JE.

JPEN J Parenter Enteral Nutr. 2019 Feb;43(2):263-270. doi: 10.1002/jpen.1316. Epub 2018 Jul 23.


Lipidomic Profiling Links the Fanconi Anemia Pathway to Glycosphingolipid Metabolism in Head and Neck Cancer Cells.

Zhao X, Brusadelli MG, Sauter S, Butsch Kovacic M, Zhang W, Romick-Rosendale LE, Lambert PF, Setchell KDR, Wells SI.

Clin Cancer Res. 2018 Jun 1;24(11):2700-2709. doi: 10.1158/1078-0432.CCR-17-3686. Epub 2018 Mar 12.


The history and basic science development of soy isoflavones.

Setchell KDR.

Menopause. 2017 Dec;24(12):1338-1350. doi: 10.1097/GME.0000000000001018. Review.


Oral Cholic Acid Is Efficacious and Well Tolerated in Patients With Bile Acid Synthesis and Zellweger Spectrum Disorders.

Heubi JE, Bove KE, Setchell KDR.

J Pediatr Gastroenterol Nutr. 2018 Feb;66(2):e57-e59. doi: 10.1097/MPG.0000000000001764. No abstract available.


Severe Neonatal Cholestasis in Cerebrotendinous Xanthomatosis: Genetics, Immunostaining, Mass Spectrometry.

Gong JY, Setchell KDR, Zhao J, Zhang W, Wolfe B, Lu Y, Lackner K, Knisely AS, Wang NL, Hao CZ, Zhang MH, Wang JS.

J Pediatr Gastroenterol Nutr. 2017 Nov;65(5):561-568. doi: 10.1097/MPG.0000000000001730.


Bile Acid Synthesis Disorders in Arabs: A 10-year Screening Study.

Al-Hussaini AA, Setchell KDR, AlSaleem B, Heubi JE, Lone K, Davit-Spraul A, Jacquemin E.

J Pediatr Gastroenterol Nutr. 2017 Dec;65(6):613-620. doi: 10.1097/MPG.0000000000001734.


A convenient approach to facilitate monitoring Gaucher disease progression and therapeutic response.

Zhang W, Oehrle M, Prada CE, Schwartz IVD, Chutipongtanate S, Wattanasirichaigoon D, Inskeep V, Dai M, Pan D, Sun Y, Setchell KDR.

Analyst. 2017 Sep 8;142(18):3380-3387. doi: 10.1039/c7an00938k.


Tissue Localization of Glycosphingolipid Accumulation in a Gaucher Disease Mouse Brain by LC-ESI-MS/MS and High-Resolution MALDI Imaging Mass Spectrometry.

Jones EE, Zhang W, Zhao X, Quiason C, Dale S, Shahidi-Latham S, Grabowski GA, Setchell KDR, Drake RR, Sun Y.

SLAS Discov. 2017 Dec;22(10):1218-1228. doi: 10.1177/2472555217719372. Epub 2017 Jul 17.


Oral Cholic Acid Is Efficacious and Well Tolerated in Patients With Bile Acid Synthesis and Zellweger Spectrum Disorders.

Heubi JE, Bove KE, Setchell KDR.

J Pediatr Gastroenterol Nutr. 2017 Sep;65(3):321-326. doi: 10.1097/MPG.0000000000001657.


Will the real bile acid sulfotransferase please stand up? Identification of Sult2a8 as a major hepatic bile acid sulfonating enzyme in mice.

Dawson PA, Setchell KDR.

J Lipid Res. 2017 Jun;58(6):1033-1035. doi: 10.1194/jlr.C077420. Epub 2017 Apr 28. No abstract available.


Hepatic MDR3 expression impacts lipid homeostasis and susceptibility to inflammatory bile duct obstruction in neonates.

Carey AN, Zhang W, Setchell KDR, Simmons JR, Shi T, Lages CS, Mullen M, Carroll K, Karns R, Bessho K, Sheridan R, Zhao X, Weber SN, Miethke AG.

Pediatr Res. 2017 Jul;82(1):122-132. doi: 10.1038/pr.2017.78. Epub 2017 May 3.


Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.

Pandey MK, Burrow TA, Rani R, Martin LJ, Witte D, Setchell KD, Mckay MA, Magnusen AF, Zhang W, Liou B, Köhl J, Grabowski GA.

Nature. 2017 Mar 2;543(7643):108-112. doi: 10.1038/nature21368. Epub 2017 Feb 22.


Fibroblast growth factor 21 correlates with weight loss after vertical sleeve gastrectomy in adolescents.

Khan FH, Shaw L, Zhang W, Salazar Gonzalez RM, Mowery S, Oehrle M, Zhao X, Jenkins T, Setchell KD, Inge TH, Kohli R.

Obesity (Silver Spring). 2016 Nov;24(11):2377-2383. doi: 10.1002/oby.21658. Epub 2016 Sep 12.


Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease.

Jian J, Tian QY, Hettinghouse A, Zhao S, Liu H, Wei J, Grunig G, Zhang W, Setchell KDR, Sun Y, Overkleeft HS, Chan GL, Liu CJ.

EBioMedicine. 2016 Nov;13:212-224. doi: 10.1016/j.ebiom.2016.10.010. Epub 2016 Oct 24.


Stable-Isotope Dilution HPLC-Electrospray Ionization Tandem Mass Spectrometry Method for Quantifying Hydroxyurea in Dried Blood Samples.

Marahatta A, Megaraj V, McGann PT, Ware RE, Setchell KD.

Clin Chem. 2016 Dec;62(12):1593-1601. Epub 2016 Sep 30.


Inhibition of ileal bile acid uptake protects against nonalcoholic fatty liver disease in high-fat diet-fed mice.

Rao A, Kosters A, Mells JE, Zhang W, Setchell KD, Amanso AM, Wynn GM, Xu T, Keller BT, Yin H, Banton S, Jones DP, Wu H, Dawson PA, Karpen SJ.

Sci Transl Med. 2016 Sep 21;8(357):357ra122. doi: 10.1126/scitranslmed.aaf4823.


Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.

Liou B, Peng Y, Li R, Inskeep V, Zhang W, Quinn B, Dasgupta N, Blackwood R, Setchell KD, Fleming S, Grabowski GA, Marshall J, Sun Y.

Hum Mol Genet. 2016 Dec 1;25(23):5126-5141. doi: 10.1093/hmg/ddw322.


Combination Therapy of All-Trans Retinoic Acid With Ursodeoxycholic Acid in Patients With Primary Sclerosing Cholangitis: A Human Pilot Study.

Assis DN, Abdelghany O, Cai SY, Gossard AA, Eaton JE, Keach JC, Deng Y, Setchell KD, Ciarleglio M, Lindor KD, Boyer JL.

J Clin Gastroenterol. 2017 Feb;51(2):e11-e16. doi: 10.1097/MCG.0000000000000591.


Long-Term Ursodeoxycholic Acid Therapy Does Not Alter Lithocholic Acid Levels in Patients with Cystic Fibrosis with Associated Liver Disease.

Colombo C, Crosignani A, Alicandro G, Zhang W, Biffi A, Motta V, Corti F, Setchell KDR.

J Pediatr. 2016 Oct;177:59-65.e1. doi: 10.1016/j.jpeds.2016.05.008. Epub 2016 Jun 11.


Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model.

Dasgupta N, Xu YH, Li R, Peng Y, Pandey MK, Tinch SL, Liou B, Inskeep V, Zhang W, Setchell KD, Keddache M, Grabowski GA, Sun Y.

Hum Mol Genet. 2015 Dec 15;24(24):7031-48. doi: 10.1093/hmg/ddv404. Epub 2015 Sep 29.


Cognitive Effects of Soy Isoflavones in Patients with Alzheimer's Disease.

Gleason CE, Fischer BL, Dowling NM, Setchell KD, Atwood CS, Carlsson CM, Asthana S.

J Alzheimers Dis. 2015;47(4):1009-19. doi: 10.3233/JAD-142958.


Let the bile flow!

Kohli R, Setchell KD.

Hepatology. 2015 Dec;62(6):1870. doi: 10.1002/hep.28244. Epub 2015 Oct 17. No abstract available.


Differential Requirements for L-Citrulline and L-Arginine during Antimycobacterial Macrophage Activity.

Rapovy SM, Zhao J, Bricker RL, Schmidt SM, Setchell KD, Qualls JE.

J Immunol. 2015 Oct 1;195(7):3293-300. doi: 10.4049/jimmunol.1500800. Epub 2015 Aug 26.


Pharmacological inhibition of apical sodium-dependent bile acid transporter changes bile composition and blocks progression of sclerosing cholangitis in multidrug resistance 2 knockout mice.

Miethke AG, Zhang W, Simmons J, Taylor AE, Shi T, Shanmukhappa SK, Karns R, White S, Jegga AG, Lages CS, Nkinin S, Keller BT, Setchell KD.

Hepatology. 2016 Feb;63(2):512-23. doi: 10.1002/hep.27973. Epub 2015 Aug 21.


Bile Acid Signaling: Mechanism for Bariatric Surgery, Cure for NASH?

Kohli R, Myronovych A, Tan BK, Salazar-Gonzalez RM, Miles L, Zhang W, Oehrle M, Sandoval DA, Ryan KK, Seeley RJ, Setchell KD.

Dig Dis. 2015;33(3):440-6. doi: 10.1159/000371699. Epub 2015 May 27. Review.


Tandem mass spectrometric determination of atypical 3β-hydroxy-Δ5-bile acids in patients with 3β-hydroxy-Δ5-C27-steroid oxidoreductase deficiency: application to diagnosis and monitoring of bile acid therapeutic response.

Zhang W, Jha P, Wolfe B, Gioiello A, Pellicciari R, Wang J, Heubi J, Setchell KD.

Clin Chem. 2015 Jul;61(7):955-63. doi: 10.1373/clinchem.2015.238238. Epub 2015 Apr 30.


Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.

Sun Y, Florer J, Mayhew CN, Jia Z, Zhao Z, Xu K, Ran H, Liou B, Zhang W, Setchell KD, Gu J, Grabowski GA.

PLoS One. 2015 Mar 30;10(3):e0118771. doi: 10.1371/journal.pone.0118771. eCollection 2015.


S-equol: a potential nonhormonal agent for menopause-related symptom relief.

Utian WH, Jones M, Setchell KD.

J Womens Health (Larchmt). 2015 Mar;24(3):200-8. doi: 10.1089/jwh.2014.5006. Epub 2015 Feb 18.


Corrigendum: The tumour suppressor LKB1 regulates myelination through mitochondrial metabolism.

Pooya S, Liu X, Kumar VB, Anderson J, Imai F, Zhang W, Ciraolo G, Ratner N, Setchell KD, Yoshida Y, Jankowski MP, Dasgupta B.

Nat Commun. 2015 Jan 9;6:6075. doi: 10.1038/ncomms7075. No abstract available.


Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.

Barnes S, Xu YH, Zhang W, Liou B, Setchell KD, Bao L, Grabowski GA, Sun Y.

PLoS One. 2014 Dec 31;9(12):e116023. doi: 10.1371/journal.pone.0116023. eCollection 2014.


The role of small heterodimer partner in nonalcoholic fatty liver disease improvement after sleeve gastrectomy in mice.

Myronovych A, Salazar-Gonzalez RM, Ryan KK, Miles L, Zhang W, Jha P, Wang L, Setchell KD, Seeley RJ, Kohli R.

Obesity (Silver Spring). 2014 Nov;22(11):2301-11. doi: 10.1002/oby.20890.


The tumour suppressor LKB1 regulates myelination through mitochondrial metabolism.

Pooya S, Liu X, Kumar VB, Anderson J, Imai F, Zhang W, Ciraolo G, Ratner N, Setchell KD, Yoshida Y, Jankowski MP, Dasgupta B.

Nat Commun. 2014 Sep 26;5:4993. doi: 10.1038/ncomms5993. Erratum in: Nat Commun. 2015;6:6075. Yutaka, Yoshida [corrected to Yoshida, Yutaka].


CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings.

Burrow TA, Sun Y, Prada CE, Bailey L, Zhang W, Brewer A, Wu SW, Setchell KDR, Witte D, Cohen MB, Grabowski GA.

Mol Genet Metab. 2015 Feb;114(2):233-241. doi: 10.1016/j.ymgme.2014.08.011. Epub 2014 Sep 2.


Treatment of bile acid amidation defects with glycocholic acid.

Heubi JE, Setchell KD, Jha P, Buckley D, Zhang W, Rosenthal P, Potter C, Horslen S, Suskind D.

Hepatology. 2015 Jan;61(1):268-74. doi: 10.1002/hep.27401. Epub 2014 Dec 23.


Synthesis of atypical bile acids for use as investigative tools for the genetic defect of 3β-hydroxy-Δ(5)-C27-steroid oxidoreductase deficiency.

Gioiello A, Cerra B, Zhang W, Vallerini GP, Costantino G, De Franco F, Passeri D, Pellicciari R, Setchell KD.

J Steroid Biochem Mol Biol. 2014 Oct;144 Pt B:348-60. doi: 10.1016/j.jsbmb.2014.06.008. Epub 2014 Jun 19.


S-(-)equol production is developmentally regulated and related to early diet composition.

Brown NM, Galandi SL, Summer SS, Zhao X, Heubi JE, King EC, Setchell KD.

Nutr Res. 2014 May;34(5):401-9. doi: 10.1016/j.nutres.2014.03.005. Epub 2014 Apr 5.


Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.

Xu YH, Xu K, Sun Y, Liou B, Quinn B, Li RH, Xue L, Zhang W, Setchell KD, Witte D, Grabowski GA.

Hum Mol Genet. 2014 Aug 1;23(15):3943-57. doi: 10.1093/hmg/ddu105. Epub 2014 Mar 5.


Metabolism of secoisolariciresinol-diglycoside the dietary precursor to the intestinally derived lignan enterolactone in humans.

Setchell KD, Brown NM, Zimmer-Nechemias L, Wolfe B, Jha P, Heubi JE.

Food Funct. 2014 Mar;5(3):491-501. doi: 10.1039/c3fo60402k.


Phytosterols promote liver injury and Kupffer cell activation in parenteral nutrition-associated liver disease.

El Kasmi KC, Anderson AL, Devereaux MW, Vue PM, Zhang W, Setchell KD, Karpen SJ, Sokol RJ.

Sci Transl Med. 2013 Oct 9;5(206):206ra137. doi: 10.1126/scitranslmed.3006898.

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