The MID1/PP2A complex: a key to the pathogenesis of Opitz BBB/G syndrome
Article first published online: 19 MAR 2003
Copyright © 2003 Wiley Periodicals, Inc.
Volume 25, Issue 4, pages 356–366, April 2003
How to Cite
Schweiger, S. and Schneider, R. (2003), The MID1/PP2A complex: a key to the pathogenesis of Opitz BBB/G syndrome. Bioessays, 25: 356–366. doi: 10.1002/bies.10256
- Issue published online: 19 MAR 2003
- Article first published online: 19 MAR 2003
Opitz BBB/G syndrome is a monogenic disorder that is characterized by malformations of the ventral midline. Investigations into the underlying genetic defects and the pathobiochemistry of this syndrome have already shed light on the mechanisms of both the physiological and the pathological development of the ventral midline, a complicated multistep process. Moreover, these studies have revealed the ubiquitin-dependent regulation of microtubule-associated phosphatase 2A, a central mechanism in many cellular processes. In this review, we summarize recent findings and speculate upon their implications for both medical and general research. BioEssays 25:356–366, 2003. © 2003 Wiley Periodicals, Inc.