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JCI online early table of contents: May 1, 2009
Date:5/1/2009

EF="mailto:metelits@bcm.tmc.edu">metelits@bcm.tmc.edu.

View the PDF of this article at: https://www.the-jci.org/article.php?id=37869


METABOLISM: The sweet smell of success: new understanding of the pathway affected in Maple Syrup Urine Disease

Yibin Wang and colleagues, at UCLA David Geffen School of Medicine, Los Angeles, have identified, in cultured cells and mice, a protein responsible for regulating the protein complex that breaks down branched-chain amino acids (three protein building blocks that cannot be made by humans but have to be obtained from the food we eat). Mice lacking this protein (PP2Cm) exhibited similar symptoms to some forms of human Maple Syrup Urine Disease (MSUD), an inherited disorder characterized by sweet-smelling urine that leads to severe brain damage and death if untreated. The authors therefore suggest that defects in PP2Cm may be responsible for some cases of MSUD.

TITLE: Protein phosphatase 2Cm is a critical regulator of branched chain-amino acid catabolism in mice and cultured cells

AUTHOR CONTACT:
Yibin Wang
David Geffen School of Medicine, University of California, at Los Angeles, Los Angeles, California, USA.
Phone: (310) 206-5197; Fax: (310) 206-5907; E-mail: yibinwang@mednet.ucla.edu.

View the PDF of this article at: https://www.the-jci.org/article.php?id=38151


CARDIOLOGY: The fog lifts on the role for the protein Fog2 in the adult heart

The protein Fog2, which is a regulator of gene expression, is essential for embryonic development of both the heart and the blood vessels that supply the heart (coronary blood vessels), but its role in the adult heart has not been determined. However, William Pu and colleagues, at Children's Hospital Boston,
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Journal of Clinical Investigation
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