Molecular pathogenesis of alpha-1-antitrypsin deficiency-associated liver disease: a meeting review (Q36804170)

16 August 2017

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title

Molecular pathogenesis of alpha-1-antitrypsin deficiency-associated liver disease: a meeting review (English)

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16 August 2017

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4

Bruce C Trapnell

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16 August 2017

David H Perlmutter

1

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16 August 2017

Jeffrey L Brodsky

series ordinal

2

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16 August 2017

William F Balistreri

series ordinal

3

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16 August 2017

language of work or name

English

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publication date

1 May 2007

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16 August 2017

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16 August 2017

45

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1313-1323

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5

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Cellular vacuolization and apoptosis induced by hepatitis B virus large surface protein

16 August 2017

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The natural history of liver disease in alpha 1-antitrypsin deficient children

21 January 2018

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7 January 2021

The mechanism of Z alpha 1-antitrypsin accumulation in the liver

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7 January 2021

Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.

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7 January 2021

A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum

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7 January 2021

Grp78, Grp94, and Grp170 interact with alpha1-antitrypsin mutants that are retained in the endoplasmic reticulum

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7 January 2021

The integration of cell and chemical biology in protein folding

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7 January 2021

Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation.

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7 January 2021

Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins

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7 January 2021

Evolving questions and paradigm shifts in endoplasmic-reticulum-associated degradation (ERAD).

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7 January 2021

INSIG: a broadly conserved transmembrane chaperone for sterol-sensing domain proteins

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7 January 2021

Proteolysis: from the lysosome to ubiquitin and the proteasome

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7 January 2021

The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

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7 January 2021

Arrest of CFTRDeltaF508 folding

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7 January 2021

Autophagy defends cells against invading group A Streptococcus

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7 January 2021

Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity

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7 January 2021

Characterization 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.

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7 January 2021

Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease

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7 January 2021

Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

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7 January 2021

IKKbeta couples hepatocyte death to cytokine-driven compensatory proliferation that promotes chemical hepatocarcinogenesis

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7 January 2021

A high-molecular-weight complex of membrane proteins BAP29/BAP31 is involved in the retention of membrane-bound IgD in the endoplasmic reticulum

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7 January 2021

Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency

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7 January 2021

Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease

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7 January 2021

CHOP induces death by promoting protein synthesis and oxidation in the stressed endoplasmic reticulum

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7 January 2021

A selective inhibitor of eIF2alpha dephosphorylation protects cells from ER stress

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7 January 2021

Role of mitochondrial permeability transition pores in mitochondrial autophagy.

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7 January 2021

Cytokines and the pathogenesis of non-alcoholic steatohepatitis

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7 January 2021

Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death.

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7 January 2021

Activation of hepatitis B virus S promoter by a cell type-restricted IRE1-dependent pathway induced by endoplasmic reticulum stress

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7 January 2021

Therapeutic approaches to protein-misfolding diseases

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7 January 2021

The biological and chemical basis for tissue-selective amyloid disease

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7 January 2021

Prevention of transthyretin amyloid disease by changing protein misfolding energetics.

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7 January 2021

Transthyretin aggregation under partially denaturing conditions is a downhill polymerization

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7 January 2021

Trans-suppression of misfolding in an amyloid disease.

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7 January 2021

Kinetic stabilization of an oligomeric protein by a single ligand binding event

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7 January 2021

Enzyme replacement and enhancement therapies: lessons from lysosomal disorders

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7 January 2021

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

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7 January 2021

Improvement in cardiac function in the cardiac variant of Fabry's disease with galactose-infusion therapy

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7 January 2021

Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

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7 January 2021

Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy

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7 January 2021

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

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7 January 2021

Treatment of the Crigler-Najjar syndrome type I with hepatocyte transplantation

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7 January 2021

Hepatocyte transplantation as a treatment for glycogen storage disease type 1a.

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7 January 2021

Stem Cell Characteristics of Amniotic Epithelial Cells

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7 January 2021

Isolation of multipotent progenitor cells from human fetal liver capable of differentiating into liver and mesenchymal lineages

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7 January 2021

Cell competition leads to a high level of normal liver reconstitution by transplanted fetal liver stem/progenitor cells.

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7 January 2021

Immune responses to recombinant adeno-associated virus vectors: putting preclinical findings into perspective

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7 January 2021

Stable therapeutic serum levels of human alpha-1 antitrypsin (AAT) after portal vein injection of recombinant adeno-associated virus (rAAV) vectors

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7 January 2021

Intramuscular administration of recombinant adeno-associated virus 2 alpha-1 antitrypsin (rAAV-SERPINA1) vectors in a nonhuman primate model: safety and immunologic aspects

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7 January 2021

Identifiers

DOI

10.1002/HEP.21628

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16 August 2017

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