Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
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Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injuryα-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiencyCapitalizing on the autophagic response for treatment of liver disease caused by alpha-1-antitrypsin deficiency and other genetic diseasesDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulumAFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase InhibitorBarriers to inhaled gene therapy of obstructive lung diseases: A reviewNew Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.Alpha1-antitrypsin deficiency and toxic shock: a Japanese autopsy case.Aggresomes and Russell bodies. Symptoms of cellular indigestion?ERAD defects and the HFE-H63D variant are associated with increased risk of liver damages in Alpha 1-Antitrypsin DeficiencyIs heterozygous alpha-1-antitrypsin deficiency type PIZ a risk factor for primary liver carcinoma?A novel model and molecular therapy for Z alpha-1 antitrypsin deficiency.A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnoverTargeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiencyAlpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy.Increased PiZ gene frequency for alpha 1 antitrypsin in patients with genetic haemochromatosisSelective protein degradation in the yeast exocytic pathway.Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver diseaseIntracellular transport, assembly, and degradation of wild-type and disease-linked mutant gap junction proteins.Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response.α1-Antitrypsin activates protein phosphatase 2A to counter lung inflammatory responses.Molecular analysis of the gene of the alpha 1-antitrypsin deficiency variant, MnichinanTherapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesPractical genetics: alpha-1-antitrypsin deficiency and the serpinopathies.Accumulation of mutant neuroserpin precedes development of clinical symptoms in familial encephalopathy with neuroserpin inclusion bodies.A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.Alpha1-antitrypsin deficiency. 4: Molecular pathophysiologyEfficient and Targeted Transduction of Nonhuman Primate Liver With Systemically Delivered Optimized AAV3B Vectors.The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsinPotential roles of tumor suppressor genes and microsatellite instability in hepatocellular carcinogenesis in southern African blacks.Misfolding and aggregation of newly synthesized proteins in the endoplasmic reticulum.Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.Negative feedback by IRE1β optimizes mucin production in goblet cells.How early studies on secreted and membrane protein quality control gave rise to the ER associated degradation (ERAD) pathway: the early history of ERAD.Functions of autophagy in normal and diseased liver.Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.
P2860
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P2860
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
description
1989 nî lūn-bûn
@nan
1989 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1989 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1989年の論文
@ja
1989年論文
@yue
1989年論文
@zh-hant
1989年論文
@zh-hk
1989年論文
@zh-mo
1989年論文
@zh-tw
1989年论文
@wuu
name
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@ast
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@en
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@nl
type
label
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@ast
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@en
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@nl
prefLabel
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@ast
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@en
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@nl
P2093
P2860
P356
P1476
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
@en
P2093
B B Rogers
D W Bullock
F J DeMayo
J A Carlson
M J Finegold
R N Sifers
P2860
P304
P356
10.1172/JCI113999
P407
P577
1989-04-01T00:00:00Z