Setting the standards: Quality control in the secretory pathway

被引:1010
作者
Ellgaard, L [1 ]
Molinari, M [1 ]
Helenius, A [1 ]
机构
[1] Swiss Fed Inst Technol, Inst Biochem, CH-8092 Zurich, Switzerland
关键词
D O I
10.1126/science.286.5446.1882
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
A variety of quality control mechanisms operate in the endoplasmic reticulum and in downstream compartments of the secretory pathway to ensure the fidelity and regulation of protein expression during cell Life and differentiation. As a rule, only proteins that pass a stringent selection process are transported to their target organelles and compartments. If proper maturation fails, the aberrant products are degraded. Quality control improves folding efficiency by retaining proteins in the special folding environment of the endoplasmic reticulum, and it prevents harmful effects that could be caused by the deployment of incompletely folded or assembled proteins.
引用
收藏
页码:1882 / 1888
页数:7
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共 114 条
  • [61] THE SORTING RECEPTOR FOR YEAST VACUOLAR CARBOXYPEPTIDASE-Y IS ENCODED BY THE VPS10 GENE
    MARCUSSON, EG
    HORAZDOVSKY, BF
    CEREGHINO, JL
    GHARAKHANIAN, E
    EMR, SD
    [J]. CELL, 1994, 77 (04) : 579 - 586
  • [62] TRANSIENT AGGREGATION OF MAJOR HISTOCOMPATIBILITY COMPLEX CLASS-II CHAINS DURING ASSEMBLY IN NORMAL SPLEEN-CELLS
    MARKS, MS
    GERMAIN, RN
    BONIFACINO, JS
    [J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 1995, 270 (18) : 10475 - 10481
  • [63] Vesicular tubular clusters between the ER and Golgi mediate concentration of soluble secretory proteins by exclusion front COPI-coated vesicles
    Martínez-Menárguez, JA
    Geuze, HJ
    Slot, JW
    Klumperman, J
    [J]. CELL, 1999, 98 (01) : 81 - 90
  • [64] Erp1p and Erp2p, partners for Emp24p and Erv25p in a yeast p24 complex
    Marzioch, M
    Henthorn, DC
    Herrmann, JM
    Wilson, R
    Thomas, DY
    Bergeron, JJM
    Solari, RCE
    Rowley, A
    [J]. MOLECULAR BIOLOGY OF THE CELL, 1999, 10 (06) : 1923 - 1938
  • [65] The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
    Meacham, GC
    Lu, Z
    King, S
    Sorscher, E
    Tousson, A
    Cyr, DM
    [J]. EMBO JOURNAL, 1999, 18 (06) : 1492 - 1505
  • [66] Fit for life in the immune system? Surrogate L chain tests H chains that test L chains
    Melchers, F
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1999, 96 (06) : 2571 - 2573
  • [67] Interaction of BiP with the J-domain of the Sec63p component of the endoplasmic reticulum protein translocation complex
    Misselwitz, E
    Staeck, O
    Matlack, KES
    Rapoport, TA
    [J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 1999, 274 (29) : 20110 - 20115
  • [68] Glycoproteins form mixed disulphides with oxidoreductases during folding in living cells
    Molinari, M
    Helenius, A
    [J]. NATURE, 1999, 402 (6757) : 90 - 93
  • [69] MOLINARI M, UNPUB
  • [70] Expression and function of heat shock protein 47: A collagen-specific molecular chaperone in the endoplasmic reticulum
    Nagata, K
    [J]. MATRIX BIOLOGY, 1998, 16 (07) : 379 - 386