Inclusion bodies and autophagosomes

被引：13

作者：

Granell, Susana ^{[1
]}

Baldini, Giulia ^{[1
]}

机构：

[1] Univ Arkansas Med Sci, Dept Biochem & Mol Biol, Little Rock, AR 72205 USA

来源：

AUTOPHAGY | 2008年 / 4卷 / 03期

关键词：

alpha; 1-antitrypsin; inclusion bodies; ER homeostasis; ER-associated degradation; autophagy; ER storage disease;

D O I：

10.4161/auto.5605

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

A hallmark of some endoplasmic reticulum (ER)-storage diseases is the formation of inclusion bodies (IBs) that are membrane-limited. The nature and function of the IBs has started to be investigated. We have recently found that sequestration of mutated alpha 1-antitrypsin (ATZ) into IBs is a cell protective mechanism that maintains ER function. We also found that IBs are ER-derived and yet separate from the main ER and do not have markers of autophagosomes and lysosomes. We propose that formation of the IBs is a quality control mechanism that leads to storage of unwanted proteins outside the secretory pathway by a mechanism different than direct autophagosome formation from the ER.

引用

页码：375 / 377

页数：3

共 29 条

[1] ULTRASTRUCTURAL ANALYSIS OF THE AUTOPHAGIC PROCESS IN YEAST - DETECTION OF AUTOPHAGOSOMES AND THEIR CHARACTERIZATION [J].

BABA, M ;

TAKESHIGE, K ;

BABA, N ;

OHSUMI, Y .

JOURNAL OF CELL BIOLOGY, 1994, 124 (06) :903-913

[2] Autophagy counterbalances endoplasmic reticulum expansion during the unfolded protein response [J].

Bernales, Sebastian ;

McDonald, Kent L. ;

Walter, Peter .

PLOS BIOLOGY, 2006, 4 (12) :2311-2324

[3] Selective autophagy of the endoplasmic reticulum [J].

Bernales, Sebastian ;

Schuck, Sebastian ;

Walter, Peter .

AUTOPHAGY, 2007, 3 (03) :285-287

[4] Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programs [J].

Cabral, CM ;

Liu, Y ;

Moremen, KW ;

Sifers, RN .

MOLECULAR BIOLOGY OF THE CELL, 2002, 13 (08) :2639-2650

[5] RISK OF CIRRHOSIS AND PRIMARY LIVER-CANCER IN ALPHA-1-ANTITRYPSIN DEFICIENCY [J].

ERIKSSON, S ;

CARLSON, J ;

VELEZ, R .

NEW ENGLAND JOURNAL OF MEDICINE, 1986, 314 (12) :736-739

[6]

FELDMANN G, 1974, GASTROENTEROLOGY, V67, P1214

[7] Sequestration of mutated α1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function [J].

Granell, Susana ;

Baldini, Giovanna ;

Mohammad, Sameer ;

Nicolin, Vanessa ;

Narducci, Paola ;

Storrie, Brian ;

Baldini, Giulia .

MOLECULAR BIOLOGY OF THE CELL, 2008, 19 (02) :572-586

[8] Accumulation of mutant α1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFκB, and BAP31 but not the unfolded protein response [J].

Hidvegi, T ;

Schmidt, BZ ;

Hale, P ;

Perlmutter, DH .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2005, 280 (47) :39002-39015

[9] ULTRASTRUCTURAL LIVER PATHOLOGY IN PATIENTS WITH MINIMAL LIVER-DISEASE AND ALPHA-1-ANTITRYPSIN DEFICIENCY - A COMPARISON BETWEEN HETEROZYGOUS AND HOMOZYGOUS PATIENTS [J].

HULTCRANTZ, R ;

MENGARELLI, S .

HEPATOLOGY, 1984, 4 (05) :937-945

[10] A 2.6 Å structure of a serpin polymer and implications for conformational disease [J].

Huntington, JA ;

Pannu, NS ;

Hazes, B ;

Read, RJ ;

Lomas, DA ;

Carrell, RW .

JOURNAL OF MOLECULAR BIOLOGY, 1999, 293 (03) :449-455

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