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, cilt.34, sa.3, ss.160-166, 2009 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 34 Sayı: 3
  • Basım Tarihi: 2009
  • Dergi Adı: TURKISH JOURNAL OF BIOCHEMISTRY-TURK BIYOKIMYA DERGISI
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.160-166
  • Hacettepe Üniversitesi Adresli: Evet

Özet

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.