Research in Dr. Hughey’s laboratory is focused on characterization of the assembly, processing and membrane trafficking of apically expressed glycoproteins in polarized kidney epithelial cells. She uses biochemistry and electrophysiology techniques to study the function of glycosylation, palmitoylation and proteolytic processing of model proteins such as the epithelial sodium channel (ENaC), gamma-glutamyltranspeptidase and the cell surface sensor MUC1. Her recent studies have revealed that ENaC is activated by a very novel mechanism of proteolytic release of inhibitory peptides in the biosynthetic pathway and post-Golgi compartments, and in pathological states such as proteinuria (kidney) and Cystic Fibrosis (lung). Her current studies of MUC1 function in normal kidney epithelia are focused on its role in epithelial survival and recovery from acute kidney injury.
- BS, Education/Biology, University of Pittsburgh, 1972
- PhD, Biochemistry, University of Pittsburgh School of Medicine, 1976
Education & Training
Al-Bataineh MM, Kinlough CL, Mi Z, Jackson EK, Mutchler SM, Emlet DR, Kellum JA, Hughey RP. KIM-1-mediated anti-inflammatory activity is preserved by MUC1 induction in the proximal tubule during ischemia-reperfusion injury. Am J Physiol Renal Physiol. 2021 Aug 1;321(2):F135-F148. PMID: 34151589.
Kleyman TR, Kashlan OB, Hughey RP. Epithelial Na+ Channel Regulation by Extracellular and Intracellular Factors. Annu Rev Physiol. 2018 Feb 10;80:263-281. PMID: 29120692.
Mukherjee A, Wang Z, Kinlough CL, Poland PA, Marciszyn AL, Montalbetti N, Carattino MD, Butterworth MB, Kleyman TR, Hughey RP. Specific Palmitoyltransferases Associate with and Activate the Epithelial Sodium Channel. J Biol Chem. 2017 Mar 10;292(10):4152-4163. PMID: 28154191.
Al-Bataineh MM, Kinlough CL, Poland PA, Pastor-Soler NM, Sutton TA, Mang HE, Bastacky SI, Gendler SJ, Madsen CS, Singh S, Monga SP, Hughey RP. Muc1 enhances the β-catenin protective pathway during ischemia-reperfusion injury. Am J Physiol Renal Physiol. 2016 Mar 15;310(6):F569-79. PMID: 26739894.
Pastor-Soler NM, Sutton TA, Mang HE, Kinlough CL, Gendler SJ, Madsen CS, Bastacky SI, Ho J, Al-Bataineh MM, Hallows KR, Singh S, Monga SP, Kobayashi H, Haase VH, Hughey RP. Muc1 is protective during kidney ischemia-reperfusion injury. Am J Physiol Renal Physiol. 2015 Jun 15;308(12):F1452-62. PMID: 25925251.
Mukherjee A, Mueller GM, Kinlough CL, Sheng N, Wang Z, Mustafa SA, Kashlan OB, Kleyman TR, Hughey RP. Cysteine palmitoylation of the γ subunit has a dominant role in modulating activity of the epithelial sodium channel. J Biol Chem. 2014 May 16;289(20):14351-9. PMID: 24692558.
Hanisch FG, Kinlough CL, Staubach S, Hughey RP. MUC1 membrane trafficking: protocols for assessing biosynthetic delivery, endocytosis, recycling, and release through exosomes. Methods Mol Biol. 2012;842:123-40. PMID: 22259133.
Kinlough CL, Poland PA, Gendler SJ, Mattila PE, Mo D, Weisz OA, Hughey RP. Core-glycosylated mucin-like repeats from MUC1 are an apical targeting signal. J Biol Chem. 2011 Nov 11;286(45):39072-81. PMID: 21937430.
Hughey RP, Carattino MD, Kleyman TR. Role of proteolysis in the activation of epithelial sodium channels. Curr Opin Nephrol Hypertens. 2007 Sep;16(5):444-50. PMID: 17693760.
Hughey RP, Bruns JB, Kinlough CL, Harkleroad KL, Tong Q, Carattino MD, Johnson JP, Stockand JD, Kleyman TR. Epithelial sodium channels are activated by furin-dependent proteolysis. J Biol Chem. 2004 Apr 30;279(18):18111-4. PMID: 15007080.
