Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy.
about
Alpha-1 antitrypsin deficiency: a commonly overlooked cause of lung diseaseAlpha 1-antitrypsin binds to and interferes with functionality of EspB from atypical and typical enteropathogenic Escherichia coli strainsCharacterization of an ERAD gene as VPS30/ATG6 reveals two alternative and functionally distinct protein quality control pathways: one for soluble Z variant of human alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ.Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injuryExpression of the secretory leukoprotease inhibitor gene in epithelial cellsCollaborative interactions between neutrophil elastase and metalloproteinases in extracellular matrix degradation in three-dimensional collagen gelsImmune-directed therapy for type 1 diabetes at the clinical level: the Immune Tolerance Network (ITN) experienceDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyChallenges and Prospects for Alpha-1 Antitrypsin Deficiency Gene TherapyWhat is Pi (proteinase inhibitor) null or PiQO?: a problem highlighted by the alpha 1 antitrypsin Mheerlen mutationAlpha1-antitrypsin deficiency and toxic shock: a Japanese autopsy case.Differential regulation of gene activity and chromatin structure within the human serpin gene cluster at 14q32.1 in macrophage microcell hybrids.Exogenous alpha 1-antitrypsin down-regulates SERPINA1 expressionSerine proteinase inhibitor therapy in alpha(1)-antitrypsin inhibitor deficiency and cystic fibrosis.Emphysema mediated by lung overexpression of ADAM10.A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency.Crucial residues in the carboxy-terminal end of C1 inhibitor revealed by pathogenic mutants impaired in secretion or function.Modulation of secretory leukoprotease inhibitor gene expression in human bronchial epithelial cells by phorbol ester.Targeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiencyIdentification of a rare p.G320R alpha-1-antitrypsin variant in emphysema and lung cancer patientsEpidemiology and carcinogenesis of hepatocellular carcinoma.Insights into the pathogenesis and treatment of cancer from inborn errors of metabolism.Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytesAlpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes.Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesUnderstanding and treating some genetic diseases.A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.Quantitative differential proteomics of yeast extracellular matrix: there is more to it than meets the eye.Exercise Capacity and Response to Training Quantitative Trait Loci in a NZW X 129S1 Intercross and Combined Cross Analysis of Inbred Mouse Strains.Identification and DNA sequence analysis of 15 new alpha 1-antitrypsin variants, including two PI*Q0 alleles and one deficient PI*M allele.Hepatic gene therapy: adenovirus enhancement of receptor-mediated gene delivery and expression in primary hepatocytes.Aralast: a new alpha1-protease inhibitor for treatment of alpha-antitrypsin deficiency.Alpha-1-antitrypsin suppresses oxidative stress in preeclampsia by inhibiting the p38MAPK signaling pathway: An in vivo and in vitro study.A family of serine proteases expressed exclusively in myelo-monocytic cells specifically processes the nuclear factor-kappa B subunit p65 in vitro and may impair human immunodeficiency virus replication in these cells.Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.Chemical, biochemical, pharmacokinetic, and biological properties of L-680,833: a potent, orally active monocyclic beta-lactam inhibitor of human polymorphonuclear leukocyte elastase.The design of a new truncated and engineered alpha1-antitrypsin based on theoretical studies: an antiprotease therapeutics for pulmonary diseases.Disorders of protein misfolding: alpha-1-antitrypsin deficiency as prototype.Multilineage transduction of resident lung cells in vivo by AAV2/8 for α1-antitrypsin gene therapy.
P2860
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P2860
Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy.
description
1990 nî lūn-bûn
@nan
1990 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
1990 թվականի մայիսին հրատարակված գիտական հոդված
@hy
1990年の論文
@ja
1990年論文
@yue
1990年論文
@zh-hant
1990年論文
@zh-hk
1990年論文
@zh-mo
1990年論文
@zh-tw
1990年论文
@wuu
name
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@ast
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@en
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@nl
type
label
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@ast
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@en
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@nl
prefLabel
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@ast
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@en
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@nl
P2860
P356
P1476
Alpha 1-antitrypsin deficiency ...... is and strategies for therapy.
@en
P2093
Crystal RG
P2860
P304
P356
10.1172/JCI114578
P407
P577
1990-05-01T00:00:00Z