JCI early table of contents for May 8, 2013
Gene replacement in pigs ameliorates cystic fibrosis-associated intestinal obstruction
Cystic fibrosis (CF) is caused by mutations in CFTR and is characterized by dysfunction of the lungs, liver, pancreas, and intestines. Approximately 15% of babies with CF are born with an obstruction of the small intestine known as meconium ileus, frequently the first sign of CF. Unlike in humans, meconium ileus occurs in 100% of newborn CF pigs. In this issue of the Journal of Clinical Investigation, Michael Welsh and colleagues at the University of Iowa demonstrate that transgenic expression of normal CFTR in the intestine of CF pigs alleviated meconium ileus. Over time, the pigs still exhibited other manifestations of CF, including liver and lung disease, reduced weight gain, and pancreatic destruction. These findings provide insight into the pathophysiology of CF and indicate that tissue-specific, partial gene replacement can ameliorate intestinal symptoms of CF.
TITLE:
Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs AUTHOR CONTACT:
Michael Welsh
Howard Hughes Medical Institute, Iowa City, IA, USA
Phone: 319-335-7619; Fax: 319-335-7623; E-mail: michael-welsh@uiowa.edu View this article at: http://www.jci.org/articles/view/68867?key=d303e72197e1b7e96749 Alzheimer's disease is associated with removal of the synaptic protein ADAM10 Alzheimer's disease is characterized by the accumulation of neurotoxic β-amyloid peptide (Aβ). ADAM10, a protein that resides in the neural synapses, has previously been shown to prevent the formation of Aβ. In this issue of the Journal of Clinical Investigation, Monica Di Luca and colleagues at the University of Milan in Milan, Italy, report that ADAM10 is removed from synapses through association with the protein AP2. Strikingly, the association between ADAM10 and AP2 was increased in human brain homogenates from Alzheimer's disease (AD) patients compared to healthy controls. Neuronal activity was shown to influence the level and activity of ADAM10 in synapses and its association with AP2. These studies identify pathological mechanisms associated with AD that control the localization of proteins at the synapse. TITLE:
Endocytosis of synaptic ADAM10 in neuronal plasticity and Alzheimer's disease AUTHOR CONTACT:
Monica Di Luca
Università degli Studi di Milano, Milano, ITA
Phone: +39-02-50318374; E-mail: monica.diluca@unimi.it View this article at: http://www.jci.org/articles/view/65401?key=ae8059e94294f112e5b2 ALSO IN THIS ISSUE TITLE:
BMPR2 is required for postimplantation uterine function and pregnancy maintenance AUTHOR CONTACT:
Martin Matzuk
Baylor College of Medicine, Houston, TX, USA
Phone: +1-713-798-6451; Fax: +1-713-798-5838; E-mail: mmatzuk@bcm.edu View this article at: http://www.jci.org/articles/view/65710?key=d529ae09c7c517ba35fa TITLE:
CXCR4 downregulation of let-7a drives chemo-resistance in acute myeloid leukemia AUTHOR CONTACT:
Michael Andreeff
The Univ of Texas M.D. Anderson Cancer Center, Houston, TX, USA
Phone: 713-792-7260; Fax: 713-794-1903; E-mail: mandreef@mdanderson.org View this article at: http://www.jci.org/articles/view/66553?key=b22074a54a1dc1e1267b TITLE:
Endothelin-2 deficiency causes growth retardation, hypothermia, and emphysema in mice AUTHOR CONTACT:
Masashi Yanagisawa
The University of Texas Southwestern Medical Center, Dallas, TX, USA
Phone: 214-648-5082; Fax: 214-648-5068; E-mail: inikc2014@gmail.com View this article at: http://www.jci.org/articles/view/66735?key=2652019fa32d09a5cca8 TITLE:
Apolipoproteins E and AV mediate lipoprotein clearance by hepatic proteoglycans AUTHOR CONTACT:
Jeffrey Esko
University of California, San Diego, La Jolla, CA, USA
Phone: 858 822-1100; Fax: 858 534-5611; E-mail: jesko@ucsd.edu View this article at: http://www.jci.org/articles/view/67398?key=91f3f4b0ee5a85c6616e TITLE:
Nuclear receptor-microRNA circuitry links muscle fiber type to energy metabolism AUTHOR CONTACT:
Daniel P. Kelly
Sanford-Burnham Med Res Institute, Orlando, FL, USA
Phone: 407-745-2069; Fax: 407-745-2001; E-mail: dkelly@sanfordburnham.org View this article at: http://www.jci.org/articles/view/67652?key=919576486d5f1f75f662 TITLE:
Acylglycerol kinase augments JAK2-STAT3 signaling in esophageal squamous cells AUTHOR CONTACT:
Libing Song
Cancer Center, Sun Yat-sen University, Guangzhou, , CHN
Phone: +86(20)87335822; E-mail: lb.song1@gmail.com View this article at: http://www.jci.org/articles/view/68143?key=d9a47d5939e8915a57fb ### END
Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs AUTHOR CONTACT:
Michael Welsh
Howard Hughes Medical Institute, Iowa City, IA, USA
Phone: 319-335-7619; Fax: 319-335-7623; E-mail: michael-welsh@uiowa.edu View this article at: http://www.jci.org/articles/view/68867?key=d303e72197e1b7e96749 Alzheimer's disease is associated with removal of the synaptic protein ADAM10 Alzheimer's disease is characterized by the accumulation of neurotoxic β-amyloid peptide (Aβ). ADAM10, a protein that resides in the neural synapses, has previously been shown to prevent the formation of Aβ. In this issue of the Journal of Clinical Investigation, Monica Di Luca and colleagues at the University of Milan in Milan, Italy, report that ADAM10 is removed from synapses through association with the protein AP2. Strikingly, the association between ADAM10 and AP2 was increased in human brain homogenates from Alzheimer's disease (AD) patients compared to healthy controls. Neuronal activity was shown to influence the level and activity of ADAM10 in synapses and its association with AP2. These studies identify pathological mechanisms associated with AD that control the localization of proteins at the synapse. TITLE:
Endocytosis of synaptic ADAM10 in neuronal plasticity and Alzheimer's disease AUTHOR CONTACT:
Monica Di Luca
Università degli Studi di Milano, Milano, ITA
Phone: +39-02-50318374; E-mail: monica.diluca@unimi.it View this article at: http://www.jci.org/articles/view/65401?key=ae8059e94294f112e5b2 ALSO IN THIS ISSUE TITLE:
BMPR2 is required for postimplantation uterine function and pregnancy maintenance AUTHOR CONTACT:
Martin Matzuk
Baylor College of Medicine, Houston, TX, USA
Phone: +1-713-798-6451; Fax: +1-713-798-5838; E-mail: mmatzuk@bcm.edu View this article at: http://www.jci.org/articles/view/65710?key=d529ae09c7c517ba35fa TITLE:
CXCR4 downregulation of let-7a drives chemo-resistance in acute myeloid leukemia AUTHOR CONTACT:
Michael Andreeff
The Univ of Texas M.D. Anderson Cancer Center, Houston, TX, USA
Phone: 713-792-7260; Fax: 713-794-1903; E-mail: mandreef@mdanderson.org View this article at: http://www.jci.org/articles/view/66553?key=b22074a54a1dc1e1267b TITLE:
Endothelin-2 deficiency causes growth retardation, hypothermia, and emphysema in mice AUTHOR CONTACT:
Masashi Yanagisawa
The University of Texas Southwestern Medical Center, Dallas, TX, USA
Phone: 214-648-5082; Fax: 214-648-5068; E-mail: inikc2014@gmail.com View this article at: http://www.jci.org/articles/view/66735?key=2652019fa32d09a5cca8 TITLE:
Apolipoproteins E and AV mediate lipoprotein clearance by hepatic proteoglycans AUTHOR CONTACT:
Jeffrey Esko
University of California, San Diego, La Jolla, CA, USA
Phone: 858 822-1100; Fax: 858 534-5611; E-mail: jesko@ucsd.edu View this article at: http://www.jci.org/articles/view/67398?key=91f3f4b0ee5a85c6616e TITLE:
Nuclear receptor-microRNA circuitry links muscle fiber type to energy metabolism AUTHOR CONTACT:
Daniel P. Kelly
Sanford-Burnham Med Res Institute, Orlando, FL, USA
Phone: 407-745-2069; Fax: 407-745-2001; E-mail: dkelly@sanfordburnham.org View this article at: http://www.jci.org/articles/view/67652?key=919576486d5f1f75f662 TITLE:
Acylglycerol kinase augments JAK2-STAT3 signaling in esophageal squamous cells AUTHOR CONTACT:
Libing Song
Cancer Center, Sun Yat-sen University, Guangzhou, , CHN
Phone: +86(20)87335822; E-mail: lb.song1@gmail.com View this article at: http://www.jci.org/articles/view/68143?key=d9a47d5939e8915a57fb ### END