©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Biol Chem. Aug 26, 2015; 6(3): 95-109
Published online Aug 26, 2015. doi: 10.4331/wjbc.v6.i3.95
Published online Aug 26, 2015. doi: 10.4331/wjbc.v6.i3.95
Role of ZAC1 in transient neonatal diabetes mellitus and glucose metabolism
Anke Hoffmann, Dietmar Spengler, Max Planck Institute of Psychiatry, Translational Research, 80804 Munich, Germany
Author contributions: Hoffmann A and Spengler D jointly contributed to this work.
Supported by Max Planck Institute of Psychiatry.
Conflict-of-interest statement: The authors declare no conflict of interest.
Correspondence to: Dietmar Spengler, MD, Max Planck Institute of Psychiatry, Translational Research, Kraepelinstrasse 2-10, 80804 Munich, Germany. spengler@psych.mpg.de
Telephone: +49-89-30622587 Fax: +49-89-30622605
Received: April 20, 2015
Peer-review started: April 24, 2015
First decision: June 3, 2015
Revised: June 19, 2015
Accepted: July 11, 2015
Article in press: July 14, 2015
Published online: August 26, 2015
Processing time: 127 Days and 17.1 Hours
Peer-review started: April 24, 2015
First decision: June 3, 2015
Revised: June 19, 2015
Accepted: July 11, 2015
Article in press: July 14, 2015
Published online: August 26, 2015
Processing time: 127 Days and 17.1 Hours
Core Tip
Core tip: Accidents of nature leading to rare genetic diseases can provide important insights into the molecular and cellular foundations of related common diseases. Various genetic anomalies at chromosome 6q24 manifest with life-threatening transient neonatal diabetes mellitus (TNDM1). All of these genetic defects share overexpress-ion of the maternally imprinted transcriptional regulator ZAC1. Genome-wide expression profiling identified a number of downstream target genes sharing a critical role in insulin secretion, β-cell proliferation, and survival. Importantly, Zac1 overexpression in β-cells spares the effects of G-protein signaling on insulin secretion opening the prospect for tailored therapy in TNDM1 patients.