Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum (Q28257006)

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Acyl-enzyme complexes between tissue-type plasminogen activator and neuroserpin are short-lived in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Neuroserpin regulates neurite outgrowth in nerve growth factor-treated PC12 cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Impaired explorative behavior and neophobia in genetically modified mice lacking or overexpressing the extracellular serine protease inhibitor neuroserpin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Familial encephalopathy with neuroserpin inclusion bodies

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Association between conformational mutations in neuroserpin and onset and severity of dementia

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Alpha1-antitrypsin deficiency--a model for conformational diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Serpinopathies and the conformational dementias

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

21 January 2018

Conformational disease

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Aggresomes, inclusion bodies and protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Human neuroserpin (PI12): cDNA cloning and chromosomal localization to 3q26

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Proteasome-dependent endoplasmic reticulum-associated protein degradation: an unconventional route to a familiar fate

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

ER quality control: the cytoplasmic connection

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Inhibition of proteasome activities and subunit-specific amino-terminal threonine modification by lactacystin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Leupeptin, a Protease Inhibitor, Decreases Protein Degradation in Normal and Diseased Muscles

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Biochemical characterization of a neuroserpin variant associated with hereditary dementia

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Intracellular accumulation of antithrombin Morioka (C95R), a novel mutation causing type I antithrombin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200

Pathogenic α1-Antitrypsin Polymers Are Formed by Reactive Loop-β-Sheet A Linkage

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M313166200