(Q34155394)

31 July 2017

title

A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover (English)

1 reference

31 July 2017

main subject

Caenorhabditis elegans

0 references

Mark T Miedel

10

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Stephan Züchner

13

object named as

Stephan Züchner

1 reference

31 July 2017

Olivia S Long

1

1 reference

31 July 2017

Joshua A Benson

2

1 reference

31 July 2017

Joon Hyeok Kwak

3

1 reference

31 July 2017

Cliff J Luke

4

1 reference

31 July 2017

Sager J Gosai

5

1 reference

31 July 2017

Linda P O'Reilly

6

1 reference

31 July 2017

Yan Wang

7

1 reference

31 July 2017

Jie Li

8

1 reference

31 July 2017

Anne C Vetica

9

1 reference

31 July 2017

Donna B Stolz

11

1 reference

31 July 2017

Simon C Watkins

12

1 reference

31 July 2017

David H Perlmutter

14

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31 July 2017

Gary A Silverman

15

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31 July 2017

Stephen C Pak

16

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31 July 2017

publication date

16 May 2014

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31 July 2017

published in

Human Molecular Genetics

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31 July 2017

volume

23

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31 July 2017

issue

19

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31 July 2017

page(s)

5109-5122

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A pro-cathepsin L mutant is a luminal substrate for endoplasmic-reticulum-associated degradation in C. elegans

31 July 2017

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

piRNAs initiate an epigenetic memory of nonself RNA in the C. elegans germline

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Daf-2 signaling modifies mutant SOD1 toxicity in C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

CREST--a large and diverse superfamily of putative transmembrane hydrolases

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Hepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Automated high-content live animal drug screening using C. elegans expressing the aggregation prone serpin α1-antitrypsin Z

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

SID1 transmembrane family, member 2 (Sidt2): a novel lysosomal membrane protein

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Reduced IGF-1 signaling delays age-associated proteotoxicity in mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Multivesicular bodies associate with components of miRNA effector complexes and modulate miRNA activity

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Silencing by small RNAs is linked to endosomal trafficking

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Deletion of Irs2 reduces amyloid deposition and rescues behavioural deficits in APP transgenic mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

The insulin paradox: aging, proteotoxicity and neurodegeneration

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Selection and validation of a set of reliable reference genes for quantitative sod gene expression analysis in C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

5 July 2018

The C. elegans intestine

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

An intracellular serpin regulates necrosis by inhibiting the induction and sequelae of lysosomal injury

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Opposing activities protect against age-onset proteotoxicity

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

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.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Rapid single nucleotide polymorphism mapping in C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Transcriptional silencing of a transgene by RNAi in the soma of C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

FRAP analysis of binding: proper and fitting

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Analysis of binding reactions by fluorescence recovery after photobleaching

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Alpha1-antitrypsin deficiency. 1: epidemiology of alpha1-antitrypsin deficiency

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Autophagy genes are essential for dauer development and life-span extension in C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Genome-wide RNAi screening in Caenorhabditis elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

The amyloid hypothesis of Alzheimer's disease: progress and problems on the road to therapeutics

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

The rde-1 gene, RNA interference, and transposon silencing in C. elegans

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Identification of a nuclear targeting domain in the insertion between helices C and D in protease inhibitor-10.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

5 July 2018

Conformational disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Theoretical analysis of fluorescence photobleaching recovery experiments

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Characterization of α1-Antitrypsin in the Inclusion Bodies from the Liver in α1-Antitrypsin Deficiency

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

The NHX family of Na+-H+ exchangers in Caenorhabditis elegans

5 July 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Decreased insulin-receptor signaling promotes the autophagic degradation of beta-amyloid peptide in C. elegans.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Systemic RNAi in C. elegans requires the putative transmembrane protein SID-1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Loss of the putative RNA-directed RNA polymerase RRF-3 makes C. elegans hypersensitive to RNAi

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Nonparametric Estimation from Incomplete Observations

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Measurement of dynamic protein binding to chromatin in vivo, using photobleaching microscopy

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4159155

Genes Required for Systemic RNA Interference in Caenorhabditis elegans

28 October 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU235

Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response

21 January 2018

1 reference

12 December 2020

Liver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

1 reference

12 December 2020

Parental effects and phenotypic characterization of mutations that affect early development in Caenorhabditis elegans

1 reference

12 December 2020

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

1 reference

12 December 2020

Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

1 reference

12 December 2020

Regulation of Caenorhabditis elegans RNA interference by the daf-2 insulin stress and longevity signaling pathway

1 reference

12 December 2020

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

1 reference

12 December 2020

Identifiers

DOI

10.1093/HMG/DDU235

1 reference

31 July 2017

1 reference

31 July 2017

1 reference