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Genomes and Genes | S D FreedmanSummaryAffiliation: Harvard University Country: USA Publications
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Publications
Fatty acids in cystic fibrosisS D Freedman
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA
Curr Opin Pulm Med 6:530-2. 2000..In addition, DHA therapy reverses the increased neutrophil infiltration in the lungs of CF knock-out mice. Further studies are required to determine the mechanism by which CF gene mutations lead to this lipid abnormality...- Association of cystic fibrosis with abnormalities in fatty acid metabolismSteven D Freedman
Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA
N Engl J Med 350:560-9. 2004..In this study we determined whether humans with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene have a similar fatty acid defect in tissues expressing CFTR... - Characterization of LPS-induced lung inflammation in cftr-/- mice and the effect of docosahexaenoic acidSteven D Freedman
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA
J Appl Physiol 92:2169-76. 2002..In contrast, neutrophils and eicosanoids were decreased in cftr-/- but not in WT mice treated with DHA, indicating that the effects of DHA on these inflammatory parameters may be related to correction of the membrane lipid defect... - Pancreatic acinar cell dysfunction in CFTR(-/-) mice is associated with impairments in luminal pH and endocytosisS D Freedman
Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02115, USA
Gastroenterology 121:950-7. 2001.... 
A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(-/-) miceS D Freedman
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
Proc Natl Acad Sci U S A 96:13995-4000. 1999..These results strongly suggest that certain phenotypic manifestations of CF may result from remediable alterations in phospholipid-bound arachidonic acid and DHA levels...- Mechanisms to explain pancreatic dysfunction in cystic fibrosisS D Freedman
Pancreas Center, Beth Israel Deaconess Medical Center, Boston, Massachussachusetts, USA
Med Clin North Am 84:657-64, x. 2000..Any of these abnormalities alone or in combination may explain the development of pancreatic exocrine insufficiency... - Nonparallel secretion of GP-2 from exocrine pancreas implies luminal coupling between acinar and duct cellsS D Freedman
Division of Gastroenterology, Charles A Dana Research Institute, Boston, Massachusetts
Am J Physiol 267:G40-51. 1994.... - GP2, a GPI-anchored protein in the apical plasma membrane of the pancreatic acinar cell, co-immunoprecipitates with src kinases and caveolinE M Parker
The Pancreas Center, Beth Israel Deaconess Medical Center, Harvard Medical Schlool, Boston, Massachusetts 02215, USA
Pancreas 21:219-25. 2000..This suggests that in pancreatic acini, GP2 may exist in a complex with src kinases, caveolin, and an 85-kDa phosphorylated substrate to regulate endocytosis at the APM... 
The safety and efficacy of oral docosahexaenoic acid supplementation for the treatment of primary sclerosing cholangitis - a pilot studyC R Martin
Department of Neonatology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
Aliment Pharmacol Ther 35:255-65. 2012..Since CFTR dysfunction leads to altered fatty acid metabolism, specifically reduced docosahexaenoic acid (DHA), we hypothesised that DHA supplementation might be an effective therapy for patients with PSC...- GP-2/THP gene family encodes self-binding glycosylphosphatidylinositol-anchored proteins in apical secretory compartments of pancreas and kidneyS Fukuoka
Laboratory of Cell and Molecular Biology, Charles A Dana Research Institute, Thorndike Laboratory, Boston, MA 02215
Proc Natl Acad Sci U S A 89:1189-93. 1992.... - A sham-controlled trial of a 5-day course of repetitive transcranial magnetic stimulation of the unaffected hemisphere in stroke patientsFelipe Fregni
Harvard Center for Non Invasive Brain Stimulation, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, KS 452, Boston, Massachusetts 02215, USA
Stroke 37:2115-22. 2006..We therefore conducted a randomized, sham-controlled, phase II trial to evaluate whether five sessions of low-frequency rTMS can increase the magnitude and duration of these effects and whether this approach is safe... - A sham-controlled, phase II trial of transcranial direct current stimulation for the treatment of central pain in traumatic spinal cord injuryFelipe Fregni
Harvard Center for Noninvasive Brain Stimulation, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
Pain 122:197-209. 2006..We discuss potential mechanisms for pain amelioration after tDCS, such as a secondary modulation of thalamic nuclei activity... - Decreased peroxisome proliferator activated receptor alpha is associated with bile duct injury in cystic fibrosis transmembrane conductance regulator-/- miceHarpreet Pall
Division of Pediatric Gastroenterology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
J Pediatr Gastroenterol Nutr 42:275-81. 2006..CONCLUSION: DSS induced bile duct injury in cftr mice is associated with a defect in PPARalpha expression, which is reversed by DHA... - A controlled clinical trial of cathodal DC polarization in patients with refractory epilepsyFelipe Fregni
Harvard Center for Noninvasive Brain Stimulation and Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, KS 452, Boston, MA 02215, U S A
Epilepsia 47:335-42. 2006..To study the effects of cathodal DC polarization in patients with refractory epilepsy and malformations of cortical development (MCDs) as indexed by seizure frequency and epileptiform EEG discharges... - Ethanol administration to cystic fibrosis knockout mice results in increased fatty acid ethyl ester productionPaola G Blanco
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
Alcohol Clin Exp Res 29:2039-45. 2005..CONCLUSION: These data show an association between CFTR dysfunction and qualitative and quantitative changes in FAEE in liver and pancreas upon ethanol exposure... - Evidence of increased flux to n-6 docosapentaenoic acid in phospholipids of pancreas from cftr-/- knockout miceMario Ollero
Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02115, USA
Metabolism 55:1192-200. 2006..This suggests an enhanced flux through the n-6 pathway beyond arachidonate. This study provides a more in-depth understanding of the fatty acid alterations found in CF, as reflected by the cftr(-/-) mouse model... - Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosisSatti Beharry
Program in Integrative Biology, The Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Am J Physiol Gastrointest Liver Physiol 292:G839-48. 2007..The potential therapeutic benefits of DHA in severe CF-associated liver disease remain to be explored... - A randomized, sham-controlled, proof of principle study of transcranial direct current stimulation for the treatment of pain in fibromyalgiaFelipe Fregni
Harvard Medical School, Boston, Massachusetts 02215, USA
Arthritis Rheum 54:3988-98. 2006.... - Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel functionHarpreet Pall
Division of Pediatric Gastroenterology, Children s Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
J Pediatr 151:255-9. 2007..To determine whether primary sclerosing cholangitis (PSC) in childhood is associated with abnormalities in cystic fibrosis transmembrane conductance regulator (CFTR)... - Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophagesCharlotte Andersson
Department of Medicine, Beth Israel Deaconess Medical Center, Boston 02215, MA, United States
J Cyst Fibros 7:68-78. 2008..Since PPARs and LXRs are important regulators of inflammation and fatty acid metabolism in macrophages, we hypothesized that these pathways are dysregulated in CF macrophages and are corrected with DHA treatment... - Fatty acid alterations and n-3 fatty acid supplementation in cystic fibrosisM Rabie Al Turkmani
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, GRB 235, 55 Fruit Street, Boston, MA 02114, USA
Prostaglandins Leukot Essent Fatty Acids 77:309-18. 2007..It is recommended that future trials include a larger population of CF patients and measure multiple clinical outcomes... - Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolismCharlotte Andersson
Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
J Lipid Res 49:1692-700. 2008..In conclusion, these data demonstrate that CFTR dysfunction is associated with altered fatty acid metabolism in cultured airway epithelial cells... - The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitisMichele D Bishop
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard School of Medicine, Boston, MA, USA
Hum Genet 118:372-81. 2005..We concluded that extensive genotyping and ion channel testing are useful to confirm or exclude the diagnosis of CF in the majority of patients with idiopathic pancreatitis... - Transcranial direct current stimulation of the unaffected hemisphere in stroke patientsFelipe Fregni
Harvard Center for Noninvasive Brain Stimulation, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA
Neuroreport 16:1551-5. 2005..These results suggest that the appropriate modulation of bihemispheric brain structures can promote motor function recovery... - Analysis of lipid abnormalities in CF miceSteven D Freedman
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
Methods Mol Med 70:517-24. 2002 - An enteral therapy containing medium-chain triglycerides and hydrolyzed peptides reduces postprandial pain associated with chronic pancreatitisJulie C Shea
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass 02215, USA
Pancreatology 3:36-40. 2003..CONCLUSIONS: A complete enteral supplement containing MCT and hydrolyzed peptides minimally increases plasma CCK levels. This therapy may be effective in reducing postprandial pain associated with chronic pancreatitis... - Acute regulation of the SLC26A3 congenital chloride diarrhoea anion exchanger (DRA) expressed in Xenopus oocytesMarina N Chernova
Molecular Medicine, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
J Physiol 549:3-19. 2003..Thus, pharmacological modulation of DRA might be a useful adjunct treatment of cystic fibrosis... - Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitisSunil Sheth
Department of Medicine, Beth Israel Deaconess Medical Center Harvard Medical School, Dana 532, 330 Brookline Avenue, Boston, MA 02215, USA
Hum Genet 113:286-92. 2003.... - Laboratory tests for the diagnosis of cystic fibrosisLan Wang
Division of Laboratory Medicine, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
Am J Clin Pathol 117:S109-15. 2002..Recently, a 2-tiered newborn screening protocol including CFTR genotyping has become popular, increasing the likelihood of early diagnosis... - Induction of colitis in cftr-/- mice results in bile duct injuryPaola G Blanco
Beth Israel Deaconess Medical Center, 330 Brookline Ave, Dana 501, Boston, MA 02215, USA
Am J Physiol Gastrointest Liver Physiol 287:G491-6. 2004..0006). Pretreatment with oral docosahexaenoic acid decreased both histological evidence of bile duct injury and serum alkaline phosphatase levels. In the setting of colitis, loss of CFTR function leads to bile duct injury... - Oral docosahexaenoic acid given to pregnant mice increases the amount of surfactant in lung and amniotic fluid in preterm fetusesPaola G Blanco
Departments of Medicine and Obstetrics and Gynecology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA
Am J Obstet Gynecol 190:1369-74. 2004..CONCLUSION: The oral administration of docosahexaenoic acid to pregnant mice during pregnancy can induce dipalmitoyl phosphatidylcholine production and secretion, which is the major lipid component of surfactant... - Analysis of cystic fibrosis gener product (CFTR) function in patients with pancreas divisum and recurrent acute pancreatitisAndres Gelrud
Division of Gastroenterology and Department of Medicine, Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA
Am J Gastroenterol 99:1557-62. 2004.... - Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is alteredMunir M Zaman
Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
Clin Diagn Lab Immunol 11:819-24. 2004..This is not a result of increased LPS receptor expression but, rather, is associated with alterations in MAPK signaling... - Lasting accelerative effects of 1 Hz and 20 Hz electrical stimulation on cortical spreading depression: relevance for clinical applications of brain stimulationFelipe Fregni
Harvard Center for Non invasive Brain Stimulation and Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, USA
Eur J Neurosci 21:2278-84. 2005..Furthermore, the effects are found to be long lasting. We believe that these findings have strong relevance to support the clinical application of therapies involving electrical stimulation for diseases of reduced cortical excitability... - Novel therapeutic approaches to the treatment of chronic abdominal visceral painFranca Patrizi
Department of Medicine and Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, USA
ScientificWorldJournal 6:472-90. 2006..This paper will present an overview of the pathophysiology and the available therapies for CAVP, focusing on the recent advances in the treatment of this pathology... - A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHAM Rabie Al-Turkmani
Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
J Lipid Res 49:1946-54. 2008..In conclusion, the evidence suggests that low LA level in cultured CF cells is due to its increased metabolism, and this increased LA metabolism is corrected by DHA supplementation... - Treatment of chronic visceral pain with brain stimulationFelipe Fregni
Ann Neurol 58:971-2. 2005 - A 21-year-old man with chronic pancreatitisMark P Callery
Harvard Medical School, and Division of General Surgery, Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA
JAMA 299:1588-94. 2008..We address the patient's concerns regarding chronic pancreatitis, including what he should expect over the next several years, whether endoscopic or surgical therapies should be considered, and whether there are any cures... - Treatment of cancer pain with noninvasive brain stimulationGisele Silva
J Pain Symptom Manage 34:342-5. 2007 - Pain in chronic pancreatitis: a salutogenic mechanism or a maladaptive brain response?Felipe Fregni
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA
Pancreatology 7:411-22. 2007..Furthermore, we consider these aspects in light of a new approach to treat visceral pain: transcranial magnetic stimulation, a noninvasive method of brain stimulation... - Docosahexaenoic acid selectively augments muscarinic stimulation of epithelial Cl- secretionIsabel Calvo Del Castillo
Department of Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
J Surg Res 110:338-43. 2003..CONCLUSION: The results suggest that DHA, which has been shown to reverse organ pathology in experimental cystic fibrosis, may selectively affect agonist-regulated transport events and other fundamental properties of epithelial cells... - Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- miceMario Ollero
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
J Cell Physiol 200:235-44. 2004..These changes may be related to the loss of function of CFTR and may be relevant to the pathogenesis of metabolic abnormalities associated with cystic fibrosis in humans... - Survey of breast-feeding practices and outcomes in the cystic fibrosis populationEliza M Parker
Department of Pediatrics, University of Massachusetts Medical School and UMass Memorial Health Care, Worcester, Massachusetts 01655, USA
Pediatr Pulmonol 37:362-7. 2004..This survey indicates that breast-feeding is not harmful to children with CF, and may be beneficial... - Potential utility of plasma fatty acid analysis in the diagnosis of cystic fibrosisIbrahim Batal
Division of Laboratory Medicine, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
Clin Chem 53:78-84. 2007..An altered distribution of fatty acids in cells and tissues is found in patients with cystic fibrosis (CF). In this study, we assessed the potential role of plasma fatty acid analysis in the diagnosis of CF...