Department of Pediatrics

Clinical Interests

Dr. Rubenstein is the Robert C. Strunk Endowed Chair for Lung and Respiratory Research and Chief of the Division of Allergy and Pulmonary Medicine, having moved to Washington University in St. Louis in 2020. He received his undergraduate degree in chemistry from The Massachusetts Institute of Technology and medical degree and PhD in pharmacology from the University of Texas Southwestern Medical Center at Dallas. He subsequently completed a residency in Pediatrics at the Children’s Hospital of Pittsburgh and a Fellowship in Pediatric Pulmonology at the Johns Hopkins School of Medicine, where he became an Instructor in Pediatrics. He then moved to the Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania where he Directed the Cystic Fibrosis Center, held the Richard B. Johnston, Jr. Endowed Chair in Pediatrics, and rose to the rank of Professor of Pediatrics with tenure. Dr. Rubenstein’s research is focused on understanding the mechanisms by which small molecules can repair the dysfunction of the most common Cystic Fibrosis-causing CFTR mutation, F508del. Through this work, he has become increasingly interested in the role of a novel chaperone of the endoplasmic reticulum, ERp29, in the biogenesis of proteins relevant to Cystic Fibrosis (CF) and other pulmonary diseases. This work has been supported by grants from the NIH, the Cystic Fibrosis Foundation including a LeRoy Matthews Physician Scientist Award, and an Established Investigator Award from the American Heart Association. He has also developed NIH- and CF Foundation-funded collaborative clinical and translational research projects focusing on CF-related diabetes, ototoxicity of aminoglycoside antibiotics in CF, and animal assisted therapy in the hospital setting, as well as participated in numerous multicenter clinical trials leading to the approval of novel therapies for CF. Dr. Rubenstein is a recognized expert in the care of people with Cystic Fibrosis, and serves as Chair of the CF Foundation Clinical Research Committee as well as a member of the CF Foundation Medical Advisory Council.

Education

• BS, Massachusetts Institute of Technology, 1984
• PhD, University of Texas Southwestern Graduate School of the Biomedical Sciences, 1990
• MD, University of Texas Southwestern Medical School, 1991

Training

• Residency in Pediatrics, The Children's Hospital of Pittsburgh, 1991 - 1994
• Fellow in Pediatric Pulmonary Diseases, The Johns Hopkins School of Medicine, 1994 - 1997

Licensure and Board Certification

• 1993 - PresMedical License, State of Pennsylvania
• 1994 - 2008American Board of Pediatrics, General Pediatrics
• 1994 - 2005Medical License, State of Maryland
• 1998 - PresAmerican Board of Pediatrics, Pediatric Pulmonology
• 2020 - PresMedical License, State of Missouri
• 2022 - PresMedical License, State of Illinois

Honors and Awards

• Texaco Philanthropic Foundation Scholarship for Academic Achievement in Chemistry, Massachusetts Institute of Technology, 1983 - 1984
• Merck Index Award for Outstanding Scholastic Achievement in Chemistry, Massachusetts Institute of Technology, 1984
• Associate Member, Sigma Xi, 1984 - 1992
• Alfred Gilman Memorial Award for Excellence in Graduate Research, Department of Pharmacology, University of Texas Southwestern Medical Center at Dallas, 1989
• Member, Alpha Omega Alpha, 1990 - Pres
• Member, Sigma Xi, 1992 - Pres
• Leroy Matthews Physician Scientist Career Development Award, Cystic Fibrosis Foundation, 1996 - 2000
• Helen B. Taussig Award for Post-doctoral Clinical Research, The Johns Hopkins School of Medicine, 1997
• Established Investigator of the American Heart Association, 2002 - 2006
• Member, Society for Pediatric Research, 2003 - Pres
• The Children’s Hospital of Philadelphia Faculty Honor Roll, 2003 - 2015
• “Faculty Teacher of the Year.” Presented by the Pediatrics Housestaff of The Children's Hospital of Philadelphia., 2006
• Dr. Milton Graub Medical Recognition Award, Delaware Valley Chapter, Cystic Fibrosis Foundation, 2018

Recent Publications view all (90)

1. Nyirjesy SC, Peleckis AJ, Eiel JN, Gallagher K, Doliba A, Tami A, Flatt AJ, De Leon DD, Hadjiliadis D, Sheikh S, Stefanovski D, Gallop R, D'Alessio DA, Rubenstein RC, Kelly A, Rickels MR. Effects of GLP-1 and GIP on Islet Function in Glucose Intolerant, Pancreatic Insufficient Cystic Fibrosis. Diabetes. 2022. PMID:35796669 
2. Downes KJ, Grim A, Shanley L, Rubenstein RC, Zuppa AF, Gastonguay MR. A Pharmacokinetic Analysis of Tobramycin in Patients Less than Five Years of Age with Cystic Fibrosis: Assessment of Target Attainment with Extended-Interval Dosing through Simulation. Antimicrob Agents Chemother. 2022;66(5):e0237721. PMCID:PMC9112897  PMID:35481751 
3. Kimple AJ, Senior BA, Naureckas ET, Gudis DA, Meyer T, Hempstead SE, Resnick HE, Albon D, Barfield W, Benoit MM, Beswick DM, Callard E, Cofer S, Downer V, Elson EC, Garinis A, Halderman A, Hamburger L, Helmick M, McCown M, McKinzie CJ, Phan H, Rodriguez K, Rubenstein RC, Severin A, Shah G, Shenoy A, Sprouse B, Virgin F, Woodworth BA, Lee SE. Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations. Int Forum Allergy Rhinol. 2022. PMID:35089650 
4. Zysman-Colman, Z., Munsar, Z., Sheikh, S., Rubenstein, R.C. and Kelly, A.. Infant BMI or Weight-for-Length and Risk of Undernutrition in Childhood Among Children with Cystic Fibrosis J Pediatr. 2021. doi:10.1016/j.jpeds.2021.11.059  PMID:34871592 
5. Kelly, A., Sheikh, S., Stefanovski, D., Peleckis, A.J., Nyirjesy, S.C., Eiel, J.N., Sidhaye, A., Localio, R., Gallop, R., De Leon, D.D., Hadjiliadis, D., Rubenstein, R.C., Rickels, M.R. Erratum to: Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance J Clin Endocrinol Metab. 2021. doi:10.1210/clinem/dgab807  PMID:34792139 
6. Garinis, A.C., Poling, G.L., Rubenstein, R.C., Konrad-Martin, D., Hullar, T., Baguley, D., Burrows, H., Chisholm, J.J., Custer, A., Driesbach Hawe, L., Hunter, L.L., Marras, T.K., Ortiz, C., Petersen, L., Steyger, P.S., Winthrop, K., Zettner, E.M., Clark, K., Hungerford, M., Vachhani, J.J., Brewer, C.C.. Clinical Considerations for Routine Auditory and Vestibular Monitoring in Patients with Cystic Fibrosis Am J Audiol. 2021;30(3S):800-809. doi:https://doi.org/10.1044/2021_AJA-21-00031  PMID:34549989 
7. Kelly A, Sheikh S, Stefanovski D, Peleckis AJ, Nyirjesy SC, Eiel JN, Sidhaye A, Localio R, Gallop R, De Leon DD, Hadjiliadis D, Rubenstein RC, Rickels MR. Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance. J Clin Endocrinol Metab. 2021;106(9):2617-2634. PMCID:PMC8660013  PMID:34406395 
8. Dalton, K.R., Ruble, K., Redding, L.E., Morris, D.O., Mueller, N.T., Thorpe, R.J.Jr., Agnew, J., Carroll, K.C., Planet, P.J., Rubenstein, R.C., Chen, A.R., Grice, E.A., Davis, M.F. . Microbial Sharing between Pediatric Patients and Therapy Dogs during Hospital Animal-Assisted Intervention Programs Microorganisms. 2021;9(5):1054. doi:doi.org/10.3390/microorganisms9051054  PMID:34068292 
9. Zysman-Colman ZN, Kilberg MJ, Harrison VS, Chesi A, Grant SFA, Mitchell J, Sheikh S, Hadjiliadis D, Rickels MR, Rubenstein RC, Kelly A. Genetic potential and height velocity during childhood and adolescence do not fully account for shorter stature in cystic fibrosis. Pediatr Res. 2021;89(3):653-659. doi:10.1038/s41390-020-0940-4  PMCID:PMC7649126  PMID:32386398 
10. Kohanski, M.A., Brown, L., Orr, M., Tan, L.H., Adappa, N.D., Palmer, J.N., Rubenstein, R.C, Cohen, N.A.. Bitter taste receptor agonists regulate epithelial two-pore potassium channels via cAMP signaling Respir Res. 2021;22(1):31:1-10. doi:10.1186/s12931-021-01631-0  PMCID:PMC7844973  PMID:33509163 
11. Kilberg MJ, Harris C, Sheikh S, Stefanovski D, Cuchel M, Kubrak C, Hadjiliadis D, Rubenstein RC, Rickels MR, Kelly A. Hypoglycemia and Islet Dysfunction following Oral Glucose Tolerance Testing in Pancreatic Insufficient Cystic Fibrosis. J Clin Endocrinol Metab. 2020;105(10):3179–3189. doi:doi.org/10.1210/clinem/dgaa448  PMID:32668452 
12. Brecker M, Khakhina S, Schubert TJ, Thompson Z, Rubenstein RC. The Probable, Possible, and Novel Functions of ERp29. Front Physiol. 2020;11:574339. PMCID:PMC7506106  PMID:33013490 
13. Sheikh S, Localio AR, Kelly A, Rubenstein RC. Abnormal glucose tolerance and the 50-gram glucose challenge test in Cystic fibrosis. J Cyst Fibros. 2020;19(5):696-699. doi:10.1016/j.jcf.2020.01.003  PMID:31974039 
14. Viviano J, Brecker M, Ferrara-Cook C, Suaud L, Rubenstein RC. Correction: ERp29 as a regulator of Insulin biosynthesis. PLoS One. 2020;15(8):e0238264. PMCID:PMC7444480  PMID:32822440 
15. Viviano J, Brecker M, Ferrara-Cook C, Suaud L, Rubenstein RC. ERp29 as a regulator of Insulin biosynthesis. PLoS One. 2020;15(5):e0233502. PMCID:PMC7239452  PMID:32433667 
16. Harris CD, Kilberg MJ, Sheikh S, Stefanovski D, Cuchel M, Kubrak C, Hadjiliadis D, Rubenstein RC, Roehrhoff Rickels M, Kelly A. MON-660 Hypoglycemia Following OGTT Is More Frequent and Pronounced in CF Compared with Controls. J Endocr Soc. 2020;4:(Suppl 1):MON-660. doi:10.1210/jendso/bvaa046.458 . PMCID:PMC7208859  
17. Dalton KR, Waite KB, Ruble K, Carroll KC, DeLone A, Frankenfield P, Serpell JA, Thorpe RJ Jr, Morris DO, Agnew J, Rubenstein RC, Davis MF. Risks associated with animal-assisted intervention programs: A literature review. Complement Ther Clin Pract. 2020;39:101145. PMCID:PMC7673300  PMID:32379677 
18. Baumgarten HD, Wright CM, Rossidis AC, Lawrence KM, Kim AG, Mejaddam AY, McGovern PE, Orr MN, Coons BE, Butt Z, Li H, Hwang G, Radu A, Brown LJ, Rubenstein RC, Peranteau WH, Davey M, Heuckeroth RO, Flake AW . The EXTrauterine Environment for Neonatal Development Supports Normal Intestinal Maturation and Development. Cell Mol Gastroenterol Hepatol. 2020;10(3):623-637. doi: 10.1016/j.jcmgh.2020.05.006. Epub 2020 May 28. PMID:32474164 
19. Kilberg MJ, Sheikh S, Stefanovski D, Kubrak C, De Leon DD, Hadjiliadis D, Rubenstein RC, Rickels MR, Kelly A. Dysregulated insulin in pancreatic insufficient cystic fibrosis with post-prandial hypoglycemia. J Cyst Fibros. 2020;19(2):310-315. PMCID:PMC7007375  PMID:31402215 
20. Hong G, Alby K, Ng SCW, Fleck V, Kubrak C, Rubenstein RC, Dorgan DJ, Kawut SM, Hadjiliadis D. The presence of Aspergillus fumigatus is associated with worse respiratory quality of life in cystic fibrosis. J Cyst Fibros. 2020;19(1):125-130. doi:10.1016/j.jcf.2019.08.008  PMID:31446018 
21. Bikard Y, Viviano J, Orr MN, Brown L, Brecker M, Jeger JL, Grits D, Suaud L, Rubenstein RC. The KDEL receptor has a role in the biogenesis and trafficking of the epithelial sodium channel (ENaC). J Biol Chem. 2019;294(48):18324-18336. PMCID:PMC6885628  PMID:31653700 
22. Cheng PC, Alexiou S, Rubenstein RC. Safety and efficacy of treatment with lumacaftor in combination with ivacaftor in younger patients with cystic fibrosis Expert Rev Respir Med. 2019;13:417-423. PMCID:PMC6482058  PMID:30929526 
23. Kelly A, De Leon DD, Sheikh S, Camburn D, Kubrak C, Peleckis AJ, Stefanovski D, Hadjiliadis D, Rickels MR, Rubenstein RC. Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor Therapy. Am J Respir Crit Care Med. 2019;199(3):342-351. PMCID:PMC6835076  PMID:30130412 
24. Nyirjesy SC, Sheikh S, Hadjiliadis D, De Leon DD, Peleckis AJ, Eiel JN, Kubrak C, Stefanovski D, Rubenstein RC, Rickels MR, Kelly A. β-Cell secretory defects are present in pancreatic insufficient cystic fibrosis with 1-hour oral glucose tolerance test glucose ≥155 mg/dL. Pediatr Diabetes. 2018;19(7):1173-1182. PMCID:PMC6364976  PMID:29885044 
25. Rubenstein RC. Editorial Focus: CFTR-dependent bicarbonate secretion by Calu-3 cells. Physiol Rep. 2018;6(10):e13691. PMCID:PMC5974715  PMID:29845767 
26. Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis. J Cyst Fibros. 2018;17(1):64-70. PMID:28866451 
27. Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J Cyst Fibros. 2018;17(1):57-63. PMID:28774749 
28. Sheikh S, Gudipaty L, De Leon DD, Hadjiliadis D, Kubrak C, Rosenfeld NK, Nyirjesy SC, Peleckis AJ, Malik S, Stefanovski D, Cuchel M, Rubenstein RC, Kelly A, Rickels MR. Reduced β-Cell Secretory Capacity in Pancreatic-Insufficient, but Not Pancreatic-Sufficient, Cystic Fibrosis Despite Normal Glucose Tolerance. Diabetes. 2017;66(1):134-144. PMCID:PMC5204312  PMID:27495225 
29. Sheikh S, Putt ME, Forde KA, Rubenstein RC, Kelly A. Elevation of one hour plasma glucose during oral glucose tolerance testing. Pediatr Pulmonol. 2015;50(10):963-9. PMCID:PMC6910238  PMID:26087115 
30. Moss RB, Flume PA, Elborn JS, Cooke J, Rowe SM, McColley SA, Rubenstein RC, Higgins M, VX11-770-110 (KONDUCT) Study Group.. Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial. Lancet Respir Med. 2015;3(7):524-33. PMCID:PMC4641035  PMID:26070913 
31. Conrad C, Lymp J, Thompson V, Dunn C, Davies Z, Chatfield B, Nichols D, Clancy J, Vender R, Egan ME, Quittell L, Michelson P, Antony V, Spahr J, Rubenstein RC, Moss RB, Herzenberg LA, Goss CH, Tirouvanziam R. Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial. J Cyst Fibros. 2015;14(2):219-27. PMID:25228446 
32. Grumbach Y, Bikard Y, Suaud L, Chanoux RA, Rubenstein RC. ERp29 regulates epithelial sodium channel functional expression by promoting channel cleavage. Am J Physiol Cell Physiol. 2014;307(8):C701-9. PMCID:PMC4199997  PMID:24944201 
33. Millington K, Miller V, Rubenstein RC, Kelly A. Patient and parent perceptions of the diagnosis and management of cystic fibrosis-related diabetes. J Clin Transl Endocrinol. 2014;1(3):100-107. PMCID:PMC5685035  PMID:29159090 
34. Adams KM, Abraham V, Spielman D, Kolls JK, Rubenstein RC, Conner GE, Cohen NA, Kreindler JL. IL-17A induces Pendrin expression and chloride-bicarbonate exchange in human bronchial epithelial cells. PLoS One. 2014;9(8):e103263. PMCID:PMC4139276  PMID:25141009 
35. Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW, GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med. 2014;190(2):175-84. PMCID:PMC4226057  PMID:24927234 
36. Sheikh S, Zemel BS, Stallings VA, Rubenstein RC, Kelly A. Body composition and pulmonary function in cystic fibrosis. Front Pediatr. 2014;2:33. PMCID:PMC3995066  PMID:24783186 
37. Alexiou S, Rubenstein RC. Seeing is believing: imaging early lung disease in cystic fibrosis. Am J Respir Crit Care Med. 2014;189(8):883-5. PMID:24735030 
38. Alexiou S and Rubenstein RC. Seeing is believing: Imaging early lung disease in cystic fibrosis. Am J Respir Crit Care Med. 2014;189:883-5. 
39. Rubenstein RC and Kreindler JL. On preventing the extinction of the physician-scientist in pediatric pulmonology Front Pediatr. 2014;2(4):1-6. PMCID:PMC3896875  PMID:24479109 
40. Chanoux RA, Shubin CB, Robay A, Suaud L, Rubenstein RC. Hsc70 negatively regulates epithelial sodium channel trafficking at multiple sites in epithelial cells. Am J Physiol Cell Physiol. 2013;305(7):C776-87. PMCID:PMC3798670  PMID:23885065 
41. Clancy JP, Dupont L, Konstan MW, Billings J, Fustik S, Goss CH, Lymp J, Minic P, Quittner AL, Rubenstein RC, Young KR, Saiman L, Burns JL, Govan JR, Ramsey B, Gupta R, Arikace Study Group.. Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection. Thorax. 2013;68(9):818-25. PMCID:PMC3756431  PMID:23749840 
42. Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordoñez CL, Stone AJ, Olson ER, Clancy JP, VX06-770-101 Study Group.. Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. PLoS One. 2013;8(7):e66955. PMCID:PMC3724869  PMID:23922647 
43. Arkin LM, Flory JH, Shin DB, Gelfand JM, Treat JR, Allen J, Rubenstein RC, Yan AC. High prevalence of aquagenic wrinkling of the palms in patients with cystic fibrosis and association with measurable increases in transepidermal water loss. Pediatr Dermatol. 2012;29(5):560-6. PMID:22471628 
44. Chanoux RA, Rubenstein RC. Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic Fibrosis. Front Pharmacol. 2012;3:137. PMCID:PMC3398409  PMID:22822398 
45. Liu J, Lu W, Guha S, Baltazar GC, Coffey EE, Laties AM, Rubenstein RC, Reenstra WW, Mitchell CH. Cystic fibrosis transmembrane conductance regulator contributes to reacidification of alkalinized lysosomes in RPE cells. Am J Physiol Cell Physiol. 2012;303(2):C160-9. PMCID:PMC3404519  PMID:22572847 
46. Mueller GM, Yan W, Copelovitch L, Jarman S, Wang Z, Kinlough CL, Tolino MA, Hughey RP, Kleyman TR, Rubenstein RC. Multiple residues in the distal C terminus of the α-subunit have roles in modulating human epithelial sodium channel activity. Am J Physiol Renal Physiol. 2012;303(2):F220-8. PMCID:PMC3404586  PMID:22573385 
47. Chanoux RA, Robay A, Shubin CB, Kebler C, Suaud L, Rubenstein RC. Hsp70 promotes epithelial sodium channel functional expression by increasing its association with coat complex II and its exit from endoplasmic reticulum. J Biol Chem. 2012;287(23):19255-65. PMCID:PMC3365957  PMID:22496374 
48. Ramsey BW, Banks-Schlegel S, Accurso FJ, Boucher RC, Cutting GR, Engelhardt JF, Guggino WB, Karp CL, Knowles MR, Kolls JK, LiPuma JJ, Lynch S, McCray PB Jr, Rubenstein RC, Singh PK, Sorscher E, Welsh M. Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report. Am J Respir Crit Care Med. 2012;185(8):887-92. PMCID:PMC3360572  PMID:22312017 
49. Mayer-Hamblett N, Kronmal RA, Gibson RL, Rosenfeld M, Retsch-Bogart G, Treggiari MM, Burns JL, Khan U, Ramsey BW, EPIC Investigators.. Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis. Pediatr Pulmonol. 2012;47(2):125-34. PMCID:PMC3214247  PMID:21830317 
50. Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax. 2012;67(1):12-8. PMCID:PMC3746507  PMID:21825083 
51. Suaud L, Miller K, Panichelli AE, Randell RL, Marando CM, Rubenstein RC. 4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells. J Biol Chem. 2011;286(52):45083-92. PMCID:PMC3247989  PMID:22069317 
52. Suratwala D, Chan JS, Kelly A, Meltzer LJ, Gallagher PR, Traylor J, Rubenstein RC, Marcus CL. Nocturnal saturation and glucose tolerance in children with cystic fibrosis. Thorax. 2011;66(7):574-8. PMID:21273357 
53. Suaud L, Miller K, Alvey L, Yan W, Robay A, Kebler C, Kreindler JL, Guttentag S, Hubbard MJ, Rubenstein RC. ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells. J Biol Chem. 2011;286(24):21239-53. PMCID:PMC3122184  PMID:21525008 
54. Brodsky J, Dougherty S, Makani R, Rubenstein RC, Kelly A. Elevation of 1-hour plasma glucose during oral glucose tolerance testing is associated with worse pulmonary function in cystic fibrosis. Diabetes Care. 2011;34(2):292-5. PMCID:PMC3024336  PMID:21228248 
55. Rubenstein RC, Lockwood SR, Lide E, Bauer R, Suaud L, Grumbach Y. Regulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells. Am J Physiol Lung Cell Mol Physiol. 2011;300(1):L88-L101. PMCID:PMC3023291  PMID:20935229 
56. Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010;363(21):1991-2003. PMCID:PMC3148255  PMID:21083385 
57. Saiman L, Anstead M, Mayer-Hamblett N, Lands LC, Kloster M, Hocevar-Trnka J, Goss CH, Rose LM, Burns JL, Marshall BC, Ratjen F, AZ0004 Azithromycin Study Group.. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010;303(17):1707-15. PMID:20442386 
58. Liou TG, Rubenstein RC. Carrier screening, incidence of cystic fibrosis, and difficult decisions. JAMA. 2009;302(23):2595-6. PMID:20009063 
59. Das S, Smith TD, Sarma JD, Ritzenthaler JD, Maza J, Kaplan BE, Cunningham LA, Suaud L, Hubbard MJ, Rubenstein RC, Koval M. ERp29 restricts Connexin43 oligomerization in the endoplasmic reticulum. Mol Biol Cell. 2009;20(10):2593-604. PMCID:PMC2682600  PMID:19321666 
60. Kashlan OB, Mueller GM, Qamar MZ, Poland PA, Ahner A, Rubenstein RC, Hughey RP, Brodsky JL, Kleyman TR. Small heat shock protein alphaA-crystallin regulates epithelial sodium channel expression. J Biol Chem. 2007;282(38):28149-56. PMCID:PMC2361386  PMID:17664274 
61. Yan W, Spruce L, Rosenblatt MM, Kleyman TR, Rubenstein RC. Intracellular trafficking of a polymorphism in the COOH terminus of the alpha-subunit of the human epithelial sodium channel is modulated by casein kinase 1. Am J Physiol Renal Physiol. 2007;293(3):F868-76. PMID:17596527 
62. Antunes MB, Woodworth BA, Bhargave G, Xiong G, Aguilar JL, Ratner AJ, Kreindler JL, Rubenstein RC, Cohen NA. Murine nasal septa for respiratory epithelial air-liquid interface cultures. Biotechniques. 2007;43(2):195-6, 198, 200 passim. PMID:17824387 
63. Suaud L, Yan W, Carattino MD, Robay A, Kleyman TR, Rubenstein RC. Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC. Am J Physiol Cell Physiol. 2007;292(4):C1553-61. PMID:17182731 
64. Suaud L, Yan W, Rubenstein RC. Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Am J Physiol Cell Physiol. 2007;292(1):C603-11. PMID:16822950 
65. Raffini LJ, Raybagkar D, Blumenstein MS, Rubenstein RC, Manno CS. Cystic fibrosis as a risk factor for recurrent venous thrombosis at a pediatric tertiary care hospital. J Pediatr. 2006;148(5):659-64. PMID:16737881 
66. Goldfarb SB, Kashlan OB, Watkins JN, Suaud L, Yan W, Kleyman TR, Rubenstein RC. Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channels. Proc Natl Acad Sci U S A. 2006;103(15):5817-22. PMCID:PMC1458656  PMID:16585520 
67. Belkin RA, Henig NR, Singer LG, Chaparro C, Rubenstein RC, Xie SX, Yee JY, Kotloff RM, Lipson DA, Bunin GR. Risk factors for death of patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med. 2006;173(6):659-66. PMCID:PMC2662949  PMID:16387803 
68. Yan W, Suaud L, Kleyman TR, Rubenstein RC. Differential modulation of a polymorphism in the COOH terminus of the alpha-subunit of the human epithelial sodium channel by protein kinase Cdelta. Am J Physiol Renal Physiol. 2006;290(2):F279-88. PMID:16174865 
69. Rubenstein RC. Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies. Mol Diagn Ther. 2006;10(5):293-301. PMID:17022692 
70. Rubenstein RC. Novel, mechanism-based therapies for cystic fibrosis. Curr Opin Pediatr. 2005;17(3):385-92. PMID:15891431 
71. Samaha FF, Rubenstein RC, Yan W, Ramkumar M, Levy DI, Ahn YJ, Sheng S, Kleyman TR. Functional polymorphism in the carboxyl terminus of the alpha-subunit of the human epithelial sodium channel. J Biol Chem. 2004;279(23):23900-7. PMID:15069064 
72. Yan W, Samaha FF, Ramkumar M, Kleyman TR, Rubenstein RC. Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes. J Biol Chem. 2004;279(22):23183-92. PMID:15047694 
73. Rubenstein RC. Expert Panelist Opinion regarding consideration of research under HHS regulations at 45 CFR 46.407. Office for Human Research Protections, Department of Health and Human Services. United States Government.Published at http://ohrp.osophs.dhhs.gov/panels/407-02pnl/exp1.pdf 2003. 
74. Suaud L, Carattino M, Kleyman TR, Rubenstein RC. Genistein improves regulatory interactions between G551D-cystic fibrosis transmembrane conductance regulator and the epithelial sodium channel in Xenopus oocytes. J Biol Chem. 2002;277(52):50341-7. PMID:12386156 
75. Zeitlin PL, Diener-West M, Rubenstein RC, Boyle MP, Lee CK, Brass-Ernst L. Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol Ther. 2002;6(1):119-26. PMID:12095312 
76. Suaud L, Li J, Jiang Q, Rubenstein RC, Kleyman TR. Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes. J Biol Chem. 2002;277(11):8928-33. PMID:11773060 
77. Rubenstein RC, Lyons BM. Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation. Am J Physiol Lung Cell Mol Physiol. 2001;281(1):L43-51. PMID:11404244 
78. Callen A, Diener-West M, Zeitlin PL, Rubenstein RC. A simplified cyclic adenosine monophosphate-mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function. J Pediatr. 2000;137(6):849-55. PMID:11113843 
79. Wang X, Moylan B, Leopold DA, Kim J, Rubenstein RC, Togias A, Proud D, Zeitlin PL, Cutting GR. Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population. JAMA. 2000;284(14):1814-9. PMID:11025834 
80. Rubenstein RC, Zeitlin PL. Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR. Am J Physiol Cell Physiol. 2000;278(2):C259-67. PMID:10666020 
81. Weyler RT, Yurko-Mauro KA, Rubenstein R, Kollen WJ, Reenstra W, Altschuler SM, Egan M, Mulberg AE. CFTR is functionally active in GnRH-expressing GT1-7 hypothalamic neurons. Am J Physiol. 1999;277(3):C563-71. PMID:10484343 
82. Rubenstein RC, Zeitlin PL. Use of protein repair therapy in the treatment of cystic fibrosis. Curr Opin Pediatr. 1998;10(3):250-5. PMID:9716885 
83. Rubenstein RC, Zeitlin PL. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med. 1998;157(2):484-90. PMID:9476862 
84. Rubenstein RC, Zeitlin PL. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med. 1998;157(2):484-490. PMID:9476862 
85. Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J Clin Invest. 1997;100(10):2457-65. PMCID:PMC508446  PMID:9366560 
86. Rubenstein RC, McVeigh U, Flotte TR, Guggino WB, Zeitlin PL. CFTR gene transduction in neonatal rabbits using an adeno-associated virus (AAV) vector. Gene Ther. 1997;4(5):384-92. PMID:9274714 
87. McIntire SC, Rubenstein RC, Gartner JC Jr, Gilboa N, Ellis D. Acute flank pain and reversible renal dysfunction associated with nonsteroidal anti-inflammatory drug use. Pediatrics. 1993;92(3):459-60. PMID:8361805 
88. Rubenstein RC, Linder ME, Ross EM. Selectivity of the beta-adrenergic receptor among Gs, Gi's, and Go: assay using recombinant alpha subunits in reconstituted phospholipid vesicles. Biochemistry. 1991;30(44):10769-77. PMID:1657154 
89. Ross EM, Wong SK, Rubenstein RC, Higashijima T. Functional domains in the beta-adrenergic receptor. Cold Spring Harb Symp Quant Biol. 1988;53 Pt 1:499-506. PMID:2855489 
90. Rubenstein RC, Wong SK, Ross EM. The hydrophobic tryptic core of the beta-adrenergic receptor retains Gs regulatory activity in response to agonists and thiols. J Biol Chem. 1987;262(34):16655-62. PMID:2890639