Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.
作者信息
Perlmutter David H
机构信息
University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
出版信息
J Clin Invest. 2002 Dec;110(11):1579-83. doi: 10.1172/JCI16787.
Abstract
摘要
相似文献
1
Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.α1-抗胰蛋白酶缺乏症中的肝损伤：一种聚集蛋白诱导线粒体损伤。
J Clin Invest. 2002 Dec;110(11):1579-83. doi: 10.1172/JCI16787.
2
Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.α1-抗胰蛋白酶缺乏症患者肝脏中的线粒体自噬与损伤
Am J Physiol Gastrointest Liver Physiol. 2004 May;286(5):G851-62. doi: 10.1152/ajpgi.00175.2003. Epub 2003 Dec 18.
3
Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.α1-抗胰蛋白酶缺乏性肝脏中的禁食：自噬的组成性激活[校正咨询]
Am J Physiol Gastrointest Liver Physiol. 2002 Nov;283(5):G1156-65. doi: 10.1152/ajpgi.00041.2002.
4
Images in clinical medicine. Alpha1-antitrypsin deficiency.
N Engl J Med. 2000 Dec 28;343(26):1933. doi: 10.1056/NEJM200012283432605.
5
The role of autophagy in alpha-1-antitrypsin deficiency.自噬在α1抗胰蛋白酶缺乏症中的作用。
Methods Enzymol. 2011;499:33-54. doi: 10.1016/B978-0-12-386471-0.00003-1.
6
7
Liver in alpha1-antitrypsin deficiency: morphologic observations and in vitro synthesis of alpha1-antitrypsin.α1抗胰蛋白酶缺乏症中的肝脏：α1抗胰蛋白酶的形态学观察与体外合成
Pediatr Res. 1976 Jan;10(1):35-40. doi: 10.1203/00006450-197601000-00007.
8
Autophagy in liver diseases.自噬与肝脏疾病。
J Hepatol. 2010 Dec;53(6):1123-34. doi: 10.1016/j.jhep.2010.07.006. Epub 2010 Aug 1.
9
Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy.α1-抗胰蛋白酶聚合与丝氨酸蛋白酶抑制剂病：病理生物学与治疗前景
J Clin Invest. 2002 Dec;110(11):1585-90. doi: 10.1172/JCI16782.
10
Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency.自噬清除在α-1抗胰蛋白酶缺乏症中导致肝脏炎症和致癌作用的易聚集蛋白。
Cell Death Differ. 2009 Jan;16(1):39-45. doi: 10.1038/cdd.2008.103. Epub 2008 Jul 11.
引用本文的文献
1
Altered polyadenylation site usage in SERPINA1 3'UTR in response to cellular stress affects A1AT protein expression.在细胞应激反应中，SERPINA1 3'非翻译区（UTR）中多聚腺苷酸化位点使用的改变会影响α1抗胰蛋白酶（A1AT）蛋白的表达。
Sci Rep. 2025 Jul 2;15(1):23510. doi: 10.1038/s41598-025-07569-3.
2
Alpha-1 antitrypsin deficiency-associated liver disease: From understudied disorder to the poster child of genetic medicine.α-1抗胰蛋白酶缺乏症相关肝病：从研究不足的疾病到基因医学的典型代表。
Hepatol Commun. 2025 Apr 14;9(5). doi: 10.1097/HC9.0000000000000699. eCollection 2025 May 1.
3
A Review of Incidence and Related Risk Factors in the Development of Hepatocellular Carcinoma.肝细胞癌发生的发病率及相关危险因素综述
Cureus. 2023 Nov 26;15(11):e49429. doi: 10.7759/cureus.49429. eCollection 2023 Nov.
4
The Mechanism of Mitochondrial Injury in Alpha-1 Antitrypsin Deficiency Mediated Liver Disease.α-1 抗胰蛋白酶缺乏症相关肝疾病中线粒体损伤的机制。
Int J Mol Sci. 2021 Dec 9;22(24):13255. doi: 10.3390/ijms222413255.
5
ER-misfolded proteins become sequestered with mitochondria and impair mitochondrial function.内质网错误折叠的蛋白质与线粒体隔离，并损害线粒体功能。
Commun Biol. 2021 Dec 2;4(1):1350. doi: 10.1038/s42003-021-02873-w.
6
Protein Misfolding and Aggregation: The Relatedness between Parkinson's Disease and Hepatic Endoplasmic Reticulum Storage Disorders.蛋白质错误折叠和聚集：帕金森病与肝内质网贮积症的相关性。
Int J Mol Sci. 2021 Nov 18;22(22):12467. doi: 10.3390/ijms222212467.
7
Defects in Protein Folding and/or Quality Control Cause Protein Aggregation in the Endoplasmic Reticulum.内质网中蛋白质折叠和/或质量控制缺陷导致蛋白质聚集。
Prog Mol Subcell Biol. 2021;59:115-143. doi: 10.1007/978-3-030-67696-4_6.
8
Autophagy in liver diseases.肝脏疾病中的自噬
World J Hepatol. 2021 Jan 27;13(1):6-65. doi: 10.4254/wjh.v13.i1.6.
9
Hepatocellular carcinoma (HCC): Epidemiology, etiology and molecular classification.肝细胞癌（HCC）：流行病学、病因学和分子分类。
Adv Cancer Res. 2021;149:1-61. doi: 10.1016/bs.acr.2020.10.001. Epub 2020 Nov 28.
10
Alpha-1 antitrypsin deficient individuals have circulating extracellular vesicles with profibrogenic cargo.缺乏α-1 抗胰蛋白酶的个体具有富含致纤维化货物的细胞外囊泡循环。
Cell Commun Signal. 2020 Sep 4;18(1):140. doi: 10.1186/s12964-020-00648-0.
本文引用的文献
1
Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy.α1-抗胰蛋白酶聚合与丝氨酸蛋白酶抑制剂病：病理生物学与治疗前景
J Clin Invest. 2002 Dec;110(11):1585-90. doi: 10.1172/JCI16782.
2
Association of calnexin with mutant peripheral myelin protein-22 ex vivo: a basis for "gain-of-function" ER diseases.体外钙连蛋白与突变型外周髓鞘蛋白-22的关联：“功能获得性”内质网疾病的一个基础。
Proc Natl Acad Sci U S A. 2002 Jul 23;99(15):9852-7. doi: 10.1073/pnas.152621799. Epub 2002 Jul 15.
3
The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol.AAA型ATP酶Cdc48/p97及其伴侣蛋白将蛋白质从内质网转运到细胞质中。
Nature. 2001 Dec 6;414(6864):652-6. doi: 10.1038/414652a.
4
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.蛋白酶体参与肝癌衍生肝细胞内质网中突变α1-抗胰蛋白酶Z的降解。
J Biol Chem. 2001 Nov 30;276(48):44865-72. doi: 10.1074/jbc.M103703200. Epub 2001 Sep 27.
5
The mitochondrial permeability transition initiates autophagy in rat hepatocytes.线粒体通透性转变启动大鼠肝细胞的自噬。
FASEB J. 2001 Oct;15(12):2286-7. doi: 10.1096/fj.01-0206fje. Epub 2001 Aug 17.
6
A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum.一种天然存在的α1-抗胰蛋白酶非聚合突变体，其特征是在内质网中滞留时间延长。
J Biol Chem. 2001 Sep 7;276(36):33893-8. doi: 10.1074/jbc.M105226200. Epub 2001 Jun 26.
7
Mitochondria recycle Ca(2+) to the endoplasmic reticulum and prevent the depletion of neighboring endoplasmic reticulum regions.线粒体将钙离子循环回内质网，防止相邻内质网区域的钙离子耗竭。
J Biol Chem. 2001 Aug 3;276(31):29430-9. doi: 10.1074/jbc.M103274200. Epub 2001 May 17.
8
Proapoptotic BAX and BAK: a requisite gateway to mitochondrial dysfunction and death.促凋亡蛋白BAX和BAK：线粒体功能障碍和死亡的必要途径。
Science. 2001 Apr 27;292(5517):727-30. doi: 10.1126/science.1059108.
9
Aggresomes and Russell bodies. Symptoms of cellular indigestion?聚集体和拉塞尔小体。细胞消化不良的症状？
EMBO Rep. 2000 Sep;1(3):225-31. doi: 10.1093/embo-reports/kvd052.
10
Aggresomes, inclusion bodies and protein aggregation.应激颗粒、包涵体与蛋白质聚集
Trends Cell Biol. 2000 Dec;10(12):524-30. doi: 10.1016/s0962-8924(00)01852-3.
Zotero 插件