CBP Faculty - Bela Z. Schmidt, Ph.D.

Bela Z. Schmidt, Ph.D.
Research Assistant Professor
Tel: 412-383-7109
Fax: 412-648-8330
bes53@pitt.edu
PubMed pub. listing
CBP Research Group(s):
Ion Channel Regulation and Genetic Disease

Research Interest

The focus of my research has been the precognition of non-native protein molecules in the endoplasmic reticulum, and the intricate interplay between intracellular transport, retention and degradation. Most recently I have been investigating the mechanism of cysteine string protein's block on the maturation of the cystic fibrosis transmembrane regulator.

Publications

Marcus NY, Marcus RA, Schmidt BZ and Haslam DB. Contribution of the HEDJ/ERdj3 cysteine-rich domain to substrate interactions. Arc. Biochem. Biophys. published online ahead of print October 4th 2007
Scott CM, Kruse KB, Schmidt BZ, Perlmutter DH, McCracken AA, and Brodsky JL. ADD66, a Gene Involved in the Endoplasmic Reticulum Associated Degradation (ERAD) of Alpha-1-Antitrypsin-Z in Yeast, Facilitates Proteasome Activity and Assembly Mol. Biol. Cell 18: 3776-3787, 2007
Zhang H, Schmidt BZ, Sun F, Condliffe SB, Butterworth MB, Youker RT, Brodsky JL, Aridor M, Frizzell RA. Cysteine string protein monitors late steps in CFTR biogenesis. J. Biol. Chem. 2006 Apr 21;281(16):11312-21.
Hidvegi T, Schmidt BZ, Hale P, Perlmutter DH. Accumulation of mutant alpha 1 antitrypsin Z in the ER activates caspases-4 and -12, NFkappa B and BAP31 but not the unfolded protein response. J. Biol. Chem. 2005 Nov 25;280(47):39002-15.
Schmidt BZ, Perlmutter DH. Grp78, Grp94 and Grp170 Interact With Alpha-1-Antitrypsin Mutants That Are Retained in the Endoplasmic Reticulum. Am J Physiol Gastrointest Liver Physiol. 2005 Apr 21; 2005 Sep;289(3):G444-55