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JCI early table of contents for Jan. 9, 2013

Small peptide ameliorates autoimmune skin blistering disease in mice

Pemphigus vulgaris is a life-threatening autoimmune skin disease that is occurs when the body's immune system generates antibodies that target proteins in the skin known as desomogleins. Desmogleins help to form the adhesive bonds that hold skin cells together and keep the skin intact. Currently, pemphigus vulgaris is treated by long-term immune suppression; however, this can leave the patient susceptible to infection. In this issue of the Journal of Clinical Investigation, researchers led by Jens Waschke at the Institute of Anatomy and Cell Biology in Munich, Germany, report on a small peptide that blocked antibody recognition of desmogleins. Importantly, the peptide could prevent antibody-mediated skin blistering when applied topically to mice. At the cellular level, the peptide improved cell-cell adhesion and attenuated signaling pathways that are activated by antibody binding. These results suggest that this peptide could serve as a treatment option for pemphigus vulgaris.

Peptide-mediated desmoglein 3 crosslinking prevents pemphigus vulgaris autoantibody-induced skin blistering

Jens Waschke
Ludwig-Maximilians-University Munich, Munich, , DEU
Phone: 0049-89-5160-4811; Fax: 0049-89-5160-4802; E-mail:

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A new treatment for kidney disease-associated heart failure?

Chronic kidney disease (CKD) patients frequently suffer from mineral bone disorder, which causes vascular calcification and, eventually, chronic heart failure. Similar to patients with CKD, mice with low levels of the protein klotho (klotho hypomorphic mice) also develop vascular calcification and have shorter life spans compared to normal mice. In this issue of the Journal of Clinical Investigation, Florian Lang and colleagues at the University of Tbingen in Germany, found that treatment with the mineralocorticoid receptor antagonist spironolactone reduced vascular calcification in klotho hypomorphic mice and increased their life span. In a companion Attending Physician article, Darryl Quarles of the University of Tennessee discusses the implications of these findings for the treatment of CKD patients.

Spironolactone-sensitive vascular calcification and Pit-1-dependent osteoblastic differentiation in klotho-hypomorphic mice

Florian Lang
Dept. of Physiology, Tuebingen, NULL, DEU
Phone: +4970712972194; Fax: +497071295618; E-mail:

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Reducing cardiovascular mortality in chronic kidney disease: something borrowed, something new

Darryl Quarles
University of Tennessee Health Science Center, memphis, TN, USA
Phone: 901-448-1459; Fax: 901-448-1188; E-mail:

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New fusion gene contributes to glioblastoma progression

Fusion genes are common chromosomal aberrations in many cancers, and can be used as prognostic markers and drug targets in clinical practice. In this issue of the Journal of Clinical Investigation, researchers led by Matti Annala at Tampere University of Technology in Finland identified a fusion between the FGFR3 and TACC3 genes in human glioblastoma samples. The protein produced by this fusion gene promoted tumor growth and progression in a mouse model of glioblastoma, while increased expression of either of the normal genes did not alter tumor progression. Ivan Babic and Paul Mischel of the University of California, San Diego, explain in the accompanying commentary that it remains unclear how this fusion protein mediates tumorigenesis.

The tumorigenic fusion FGFR3-TACC3 escapes miR-99a regulation in glioblastoma

Matti Annala
Tampere University of Technology, Tampere, FIN
Phone: +358415079885; E-mail:

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Multiple functions of a glioblastoma fusion oncogene

Paul Mischel
UCSD, La Jolla, CA, USA
Phone: (858) 534-6080; Fax: ; E-mail:

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Type 1 diabetes patients exhibit altered cerebral metabolism during hypoglycemia

Bastiaan de Galan
Radboud University Nijmegen Medical Centre, Nijmegen, UNK, NLD
Phone: +31243618819; Fax: +31243541734; E-mail:

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Regulation of dendritic cell activation by microRNA let-7c and BLIMP1

Betty Diamond
The Feinstein Institute for Medical Research, Manhasset, NY, USA
Phone: 516-562-3830; Fax: 516-562-2953; E-mail:

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Liver acid sphingomyelinase inhibits growth of metastatic colon cancer

Yosuke Osawa
Gifu University Graduate School of Medicine, Gifu, JPN
Phone: 81-58-230-6217; Fax: 81-58-230-6218; E-mail:

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Specialized role of migratory dendritic cells in peripheral tolerance induction

Juliana Idoyaga
The Rockefeller University, New York, NY, USA
Phone: 212-327-7863; Fax: 212-327-8875; E-mail:

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Contact: Jillian Hurst
Journal of Clinical Investigation

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