Pyruvate Dehydrogenase Complex Deficiency Caused by the Ubiquitin-Proteasome System-Mediated Degradation in E1β Subunit

被引:0
|
作者
Han, Zongchao
Srivastava, Arun
Stacpoole, Peter
机构
[1] Univ Florida, Div Cellular & Mol Therapy, Coll Med, Alachua, FL USA
[2] Univ Florida, Coll Med, Gainesville, FL USA
[3] Univ Florida, Coll Med, Gen Clin Res Ctr, Gainesville, FL USA
关键词
D O I
10.1016/j.ymthe.2006.08.249
中图分类号
Q81 [生物工程学(生物技术)]; Q93 [微生物学];
学科分类号
071005 ; 0836 ; 090102 ; 100705 ;
摘要
224
引用
收藏
页码:S86 / S86
页数:1
相关论文
共 50 条
  • [22] Stabilization of the pyruvate dehydrogenase E1α subunit by dichloroacetate
    Morten, KJ
    Caky, M
    Matthews, PM
    NEUROLOGY, 1998, 51 (05) : 1331 - 1335
  • [23] E1AF degradation by a ubiquitin-proteasome pathway
    Takahashi, A
    Higashino, F
    Aoyagi, M
    Yoshida, K
    Itoh, M
    Kobayashi, M
    Totsuka, Y
    Kohgo, T
    Shindoh, M
    BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 2005, 327 (02) : 575 - 580
  • [24] PYRUVATE DEHYDROGENASE DEFICIENCY DUE TO NOVEL AND KNOWN MUTATIONS IN THE E1 ALPHA SUBUNIT
    Wibrand, F.
    Kalkanoglu, H. S.
    Dursun, A.
    Tokatli, A.
    Coskun, T.
    Lund, A. M.
    Christensen, E.
    Kibaek, M.
    Ostergaard, E.
    Moller, L. B.
    JOURNAL OF INHERITED METABOLIC DISEASE, 2005, 28 : 122 - 122
  • [25] Mutations of the E1β subunit gene (PDHB) in four families with pyruvate dehydrogenase deficiency
    Okajima, K.
    Korotchkina, L. G.
    Prasad, C.
    Rupar, T.
    Phillips, J. A., III
    Ficicloglu, C.
    Hertecant, J.
    Patel, M. S.
    Kerr, D. S.
    MOLECULAR GENETICS AND METABOLISM, 2008, 93 (04) : 371 - 380
  • [26] A therapeutic dose of doxorubicin activates ubiquitin-proteasome system-mediated proteolysis by acting on both the ubiquitination apparatus and proteasome
    Liu, Jinbao
    Zheng, Hanqiao
    Tang, Mingxin
    Ryu, Youn-Chul
    Wang, Xuejun
    AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY, 2008, 295 (06): : H2541 - H2550
  • [27] Pyruvate dehydrogenase complex deficiency caused by a novel missense point mutation (L213P) in the E1β subunit gene.
    Han, ZC
    Simpson, NE
    Constantinidis, I
    Stacpoole, PW
    MOLECULAR GENETICS AND METABOLISM, 2005, 84 (03) : 221 - 222
  • [28] PYRUVATE DEHYDROGENASE DEFICIENCY CAUSED BY A NOVEL MUTATION IN E1BETA SUBUNIT
    Makhseed, N.
    Brown, R. M.
    Hammoud, M.
    Brown, G. K.
    MOLECULAR GENETICS AND METABOLISM, 2009, 98 (1-2) : 94 - 94
  • [29] Tissue-specific pyruvate dehydrogenase deficiency in a boy with novel mutations in the E1 β subunit
    Mayr, J. A.
    Koch, J.
    Rauscher, C.
    Bernert, G.
    Sperl, W.
    JOURNAL OF INHERITED METABOLIC DISEASE, 2008, 31 : 54 - 54
  • [30] Ubiquitin proteasome system-mediated degradation of synaptic proteins: An update from the postsynaptic side
    Tsai, Nien-Pei
    BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH, 2014, 1843 (12): : 2838 - 2842