Publications
2024
β-hydroxybutyrate recapitulates the beneficial effects of ketogenic metabolic therapy in polycystic kidney disease. iScience. 2024 ;27(9):110773. .
The case for a ketogenic diet in the management of kidney disease. BMJ Open Diabetes Res Care. 2024 ;12(2):e004101. .
A Combination of ß-Hydroxybutyrate and Citrate Ameliorates Disease Progression in a Rat Model of Polycystic Kidney Disease. Am J Physiol Renal Physiol. 2024 ;326:F352-F368. .
Ketogenic Metabolic Therapy for Chronic Kidney Disease. Clinical Kidney Journal. 2024 ;17:sfad273. .
2023
2022
RESET-PKD: A pilot trial on short-term ketogenic interventions in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant. 2022 . .
Restoration of atypical protein kinase C ζ function in autosomal dominant polycystic kidney disease ameliorates disease progression. Proc Natl Acad Sci U S A. 2022 ;119(30):e2121267119. .
Pharmacological Effects of Panduratin A on Renal Cyst Development in In Vitro and In Vivo Models of Polycystic Kidney Disease. International Journal of Molecular Sciences. 2022 ;23:4328-4343. .
2021
Ketogenic Dietary Interventions in Autosomal-Dominant Polycystic Kidney Disease (ADPKD)– A Retrospective Case Series Study: First insights into Feasibility, Safety and Effects. Clinical Kidney Journal. 2021 ;15:1079-1092. .
2020
The carboxy-terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC-activation. J Cell Mol Med. 2020 ;24:14633–14638. .
The Habc domain of syntaxin 3 is a ubiquitin binding domain. Sci Rep. 2020 ;10(1):21350. .
STAT signaling in polycystic kidney disease. Cellular Signalling. 2020 ;72:109639. .
2019
Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease. J Clin Invest. 2019 ;130:4506-4522. .
https://doi.org/10.1172/JCI128503
* UCSB News Release: "Strike Three: Researchers uncover a previously unrecognized mechanism..."
* Highlighted in "ASN In the Loop" (American Society of Nephrology)
* Nature Reviews Nephrology, "Crystal deposition aids cystogenesis" (24 September 2019)
* Atta M: F1000Prime Recommendation of [Torres JA et al., J Clin Invest 2019 130]. In F1000Prime, 12 Sep 2019
* UCSB News Release: "Strike Three: Researchers uncover a previously unrecognized mechanism..."
* Highlighted in "ASN In the Loop" (American Society of Nephrology)
* Nature Reviews Nephrology, "Crystal deposition aids cystogenesis" (24 September 2019)
* Atta M: F1000Prime Recommendation of [Torres JA et al., J Clin Invest 2019 130]. In F1000Prime, 12 Sep 2019
Methods in Kidney Cell Biology, Part B. Methods Cell Biol. 2019 ;154:xiii-xiv. .
Methods in Kidney Cell Biology, Part A. Methods Cell Biol. 2019 ;153:xiii-xiv. .
Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease. Cell Metabolism. 2019 ;30:1007–1023. .
2018
Comparison of folate-conjugated rapamycin versus unconjugated rapamycin in an orthologous mouse model of polycystic kidney disease. Am J Physiol Renal Physiol. 2018 ;315:F395–F405. .
Casein kinase 1ε and 1α as novel players in polycystic kidney disease and mechanistic targets for (R)-roscovitine and (S)-CR8. Am J Physiol Renal Physiol. 2018 ;315:F57–F73. .
Identification of Targets of Interleukin-13 and Signal Transducer and Activator of Transcription-6 (STAT6) Signaling in Polycystic Kidney Disease. Am J Physiol Renal Physiol. 2018 ;315:F86–F96. .
Soluble syntaxin 3 functions as a transcriptional regulator. J Biol Chem. 2018 ;293:5478-5491. .
Emerging targeted strategies for the treatment of autosomal dominant polycystic kidney disease. Clinical Kidney Journal. 2018 ;11(suppl_1):i27–i38. .
Tracking Endocytosis and Intracellular Trafficking of Epitope-tagged Syntaxin 3 by Antibody Feeding in Live, Polarized MDCK Cells. Bio-Protocol. 2018 ;8(3):e2453. .
Are Cyst-Associated Macrophages in Polycystic Kidney Disease the Equivalent to TAMs in Cancer?. J Am Soc Nephrol. 2018 ;29:2447-2448. .
2017
2016
The SNARE Protein Syntaxin 3 Confers Specificity for Polarized Axonal Trafficking in Neurons. PLoS One. 2016 ;11(9):e0163671. .
Regulation of Polycystin-1 Function by Calmodulin Binding. PLoS One. 2016 ;11(8):e0161525. .
A mild reduction of food intake slows disease progression in an orthologous mouse model of polycystic kidney disease. Am J Physiol Renal Physiol. 2016 ;310: F726 –F731. .
2015
Bicc1 polymerization regulates the localization and silencing of bound mRNA. Mol Cell Biol. 2015 ;35:3339-3353. .
2014
The cleaved cytoplasmic tail of polycystin-1 regulates Src-dependent STAT3 activation. J Am Soc Nephrol. 2014 ;25(8):1737-48. .
2013
STAT3 signaling in polycystic kidney disease. Drug Discovery Today: Disease Mechanisms. 2013 ;10(3-4):e113-e118. .
Regulation of STATs by polycystin-1 and their role in polycystic kidney disease. JAK-STAT. 2013 ;2:0–1. .
2012
Folate-conjugated rapamycin slows progression of polycystic kidney disease. Journal of the American Society of Nephrology. 2012 ;23:1674. .
2011
Third-hit signaling in renal cyst formation. Journal of the American Society of Nephrology. 2011 ;22:793–795. .
Signal transducer and activator of transcription-6 (STAT6) inhibition suppresses renal cyst growth in polycystic kidney disease. Proceedings of the National Academy of Sciences. 2011 ;108:18067–18072. .
Polycystin-1 regulates STAT activity by a dual mechanism. Proceedings of the National Academy of Sciences. 2011 ;108:7985–7990. .
2010
Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. Journal of the American Society of Nephrology. 2010 ;21:489–497. .
Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases. Clinical Journal of the American Society of Nephrology. 2010 ;5:1312–1329. .
2009
Syntaxin specificity of aquaporins in the inner medullary collecting duct. American Journal of Physiology-Renal Physiology. 2009 ;297:F292–F300. .
2007
Polycystic kidney disease and renal injury repair: common pathways, fluid flow, and the function of polycystin-1. American Journal of Physiology-Renal Physiology. 2007 ;293:F1423–F1432. .
2006
Differing effects of microtubule depolymerizing and stabilizing chemotherapeutic agents on t-SNARE–mediated apical targeting of prostate-specific membrane antigen. Molecular Cancer Therapeutics. 2006 ;5:2468–2473. .
The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proceedings of the National Academy of Sciences. 2006 ;103:5466–5471. .
* UCSB Press Release
* Accompanying Commentary: "mTOR is out of control in polycystic kidney disease" PNAS 103, 5247–5248
* Featured on the cover and "In This Issue" of PNAS
* Featured in Nature Genetics, vol 38, p. 511 (2006) "Research Highlights: Polycystic kidney disease and mTOR"
* Featured in Nature Clinical Practice Nephrology (2006) 2, 295 "Research Highlight"
* Featured in Kidney International (2006) 69, 2122–2123 "Journal Club: Medical therapy for renal cystic disease"
* "Must Read", Faculty of 1000 - Biology
* Harris R: F1000Prime Recommendation. In F1000Prime, 18 Apr 2006
* Cook D: F1000Prime Recommendation. In F1000Prime, 27 Apr 2006
* Accompanying Commentary: "mTOR is out of control in polycystic kidney disease" PNAS 103, 5247–5248
* Featured on the cover and "In This Issue" of PNAS
* Featured in Nature Genetics, vol 38, p. 511 (2006) "Research Highlights: Polycystic kidney disease and mTOR"
* Featured in Nature Clinical Practice Nephrology (2006) 2, 295 "Research Highlight"
* Featured in Kidney International (2006) 69, 2122–2123 "Journal Club: Medical therapy for renal cystic disease"
* "Must Read", Faculty of 1000 - Biology
* Harris R: F1000Prime Recommendation. In F1000Prime, 18 Apr 2006
* Cook D: F1000Prime Recommendation. In F1000Prime, 27 Apr 2006
A dual tyrosine-leucine motif mediates myelin protein P0 targeting in MDCK cells. Glia. 2006 ;54:135–145. .
Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease. Developmental Cell. 2006 ;10:57–69. .
Regulation of mTOR by polycystin-1: is polycystic kidney disease a case of futile repair?. Cell Cycle. 2006 ;5:2425–2429. .
Apical targeting of syntaxin 3 is essential for epithelial cell polarity. The Journal of Cell Biology. 2006 ;173:937–948. .
Syntaxins 3 and 4 are concentrated in separate clusters on the plasma membrane before the establishment of cell polarity. Molecular Biology of the Cell. 2006 ;17:977–989. .
2005
Image segmentation, registration and visualization of serial MR images for therapeutic assessment of polycystic kidney disease in transgenic mice. Conf Proc IEEE Eng Med Biol Soc. . 2005 :467–469. .
2004
Regulation of nuclear functions–nucleocytoplasmic transport in context. European Journal of Cell Biology. 2004 ;83:185–192. .
Polarity proteins control ciliogenesis via kinesin motor interactions. Current Biology. 2004 ;14:1451–1461. .
2003
SNAREs and epithelial cells. Methods. 2003 ;30:191–197. .
Matrix metalloproteinase activity in urine of patients with renal cell carcinoma leads to degradation of extracellular matrix proteins: possible use as a screening assay. The Journal of Urology. 2003 ;169:1530–1534. .
Three-dimensional analysis of post-Golgi carrier exocytosis in epithelial cells. Nature Cell Biology. 2003 ;5:126–136. .
Syntaxin 2 and endobrevin are required for the terminal step of cytokinesis in mammalian cells. Developmental Cell. 2003 ;4:753–759. .
2002
Retinal pigment epithelial cells exhibit unique expression and localization of plasma membrane syntaxins which may contribute to their trafficking phenotype. Journal of Cell Science. 2002 ;115:4545–4553. .
SNARE expression and localization in renal epithelial cells suggest mechanism for variability of trafficking phenotypes. American Journal of Physiology-Renal Physiology. 2002 ;283:F1111–F1122. .
Direct interaction between Rab3b and the polymeric immunoglobulin receptor controls ligand-stimulated transcytosis in epithelial cells. Developmental Cell. 2002 ;2:219–228. .
2001
Identification of discrete classes of endosome-derived small vesicles as a major cellular pool for recycling membrane proteins. Molecular Biology of the Cell. 2001 ;12:981–995. .
2000
Intracellular redirection of plasma membrane trafficking after loss of epithelial cell polarity. Molecular Biology of the Cell. 2000 ;11:3045–3060. .
1998
Targeting of SNAP-23 and SNAP-25 in polarized epithelial cells. Journal of Biological Chemistry. 1998 ;273:3422–3430. .
The SNARE machinery is involved in apical plasma membrane trafficking in MDCK cells. The Journal of Cell Biology. 1998 ;141:1503–1513. .
A model for structural similarity between different SNARE complexes based on sequence relationships. Trends in Cell Biology. 1998 ;8:260–262. .
1997
A conserved domain is present in different families of vesicular fusion proteins: a new superfamily. Proceedings of the National Academy of Sciences. 1997 ;94:3046–3051. .
Apical targeting in polarized epithelial cells: there's more afloat than rafts. Trends in Cell Biology. 1997 ;7:393–399. .
1996
Differential localization of syntaxin isoforms in polarized Madin-Darby canine kidney cells. Molecular Biology of the Cell. 1996 ;7:2007-2018. .
1995
Regulation of protein traffic in polarized epithelial cells: the polymeric immunoglobulin receptor model. Cold Spring Harbor Symposia on Quantitative Biology. 1995 ;60:775–781. .
1994
Topology of CNS Myelin Proteolipid Protein: Evidence for the Nonenzymic Glycosylation of Extracytoplasmic Domains in Normal and Diabetic Animals. Biochemistry. 1994 ;33:10408–10415. .
1992
1990
A point mutation at the X-chromosomal proteolipid protein locus in Pelizaeus-Merzbacher disease leads to disruption of myelinogenesis. Biological Chemistry Hoppe-Seyler. 1990 ;371:1175–1184. .