Alpha-1-antitrypsin deficiency: accumulation or degradation of mutant variants within the hepatic endoplasmic reticulum (Q38784755)

6 September 2017

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title

Alpha-1-antitrypsin deficiency: accumulation or degradation of mutant variants within the hepatic endoplasmic reticulum (English)

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alpha-1-antitrypsin deficiency

6 September 2017

main subject

endoplasmic reticulum

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Sifers RN

1

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6 September 2017

Finegold MJ

series ordinal

2

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6 September 2017

Woo SL

series ordinal

3

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6 September 2017

publication date

1 November 1989

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American Journal of Respiratory Cell and Molecular Biology

6 September 2017

published in

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6 September 2017

volume

1

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6 September 2017

issue

5

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6 September 2017

page(s)

341-345

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Alpha 1 antitrypsin deficiency due to MMaltonZ phenotype: case report and family study

6 September 2017

cites work

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Complete sequence of the cDNA for human .alpha.1-antitrypsin and the gene for the S variant

21 January 2018

1 reference

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Tissue specific expression of the human alpha-1-antitrypsin gene in transgenic mice

21 January 2018

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Alpha 1-antitrypsin deficiency caused by the alpha 1-antitrypsin Nullmattawa gene. An insertion mutation rendering the alpha 1-antitrypsin gene incapable of producing alpha 1-antitrypsin

21 January 2018

1 reference

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Purification and Partial Characterization of PAS-Positive Inclusion Bodies from the Liver in Alpha1-Antitrypsin Deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1165%2FAJRCMB%2F1.5.341

Molecular basis for defective secretion of the Z variant of human alpha-1-proteinase inhibitor: secretion of variants having altered potential for salt bridge formation between amino acids 290 and 342.

21 January 2018

1 reference

https://api.crossref.org/works/10.1165%2FAJRCMB%2F1.5.341

Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function

21 January 2018

1 reference

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Disruption of the Lys-290--Glu-342 salt bridge in human alpha 1-antitrypsin does not prevent its synthesis and secretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1165%2FAJRCMB%2F1.5.341

Posttranslational association of immunoglobulin heavy chain binding protein with nascent heavy chains in nonsecreting and secreting hybridomas

21 January 2018

1 reference

https://api.crossref.org/works/10.1165%2FAJRCMB%2F1.5.341

The relationship of N-linked glycosylation and heavy chain-binding protein association with the secretion of glycoproteins

21 January 2018

1 reference

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

21 January 2018

1 reference

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Multiple tissues express alpha 1-antitrypsin in transgenic mice and man.

21 January 2018

1 reference

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21 January 2018

Identifiers

DOI

10.1165/AJRCMB/1.5.341

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6 September 2017

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