Inhibition of Endoplasmic Reticulum Associated Degradation of Mutant Pro-beta Subunits of beta-Hexosaminidase by Kifunensine and Lactacystin

SINICI I., Tropak M. B., Oezkara H. A., Mahuran D. J.

TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI, vol.34, no.3, pp.160-166, 2009 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 34 Issue: 3
  • Publication Date: 2009
  • Journal Name: TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.160-166
  • Hacettepe University Affiliated: Yes

Abstract

Objectives: We investigated the stabilizing effects of inhibiting Endoplasmic Reticulum Associated Degradation using kifunensine and lactacystin on heterologous expression of two mutant pro-forms of beta-subunit of beta-hexosaminidase, 12 bp deletion and D208N missense mutations.