(Q36816779)

16 August 2017

1 reference

reason for preferred rank

title

α 1 ‐antitrypsin deficiency and liver disease (English)

1 reference

https://api.crossref.org/works/10.1007/BF01797921

15 November 2022

Alpha 1-antitrypsin deficiency and liver disease (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1749216

alpha-1-antitrypsin deficiency

15 November 2022

main subject

1 reference

1 reference

https://api.crossref.org/works/10.1007/BF01797921

15 November 2022

author name string

McElvaney NG

2

1 reference

16 August 2017

Chang-Stroman LM

3

1 reference

16 August 2017

P. Birrer

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1749216

15 November 2022

N. G. McElvaney

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1749216

15 November 2022

L. M. Chang-Stroman

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1749216

15 November 2022

R. G. Crystal

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1749216

15 November 2022

language of work or name

1 reference

1 January 1991

1 reference

full work available at URL

16 August 2017

https://onlinelibrary.wiley.com/doi/pdf/10.1007/BF01797921

1 reference

https://api.crossref.org/works/10.1007/BF01797921

Journal of Inherited Metabolic Disease

15 November 2022

published in

1 reference

16 August 2017

volume

14

1 reference

16 August 2017

issue

4

1 reference

16 August 2017

page(s)

512-525

1 reference

Molecular basis of alpha-1-antitrypsin deficiency

16 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1007%2FBF01797921

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/10.1007%2FBF01797921

Immunohistochemistry of liver in alpha1-antitrypsin deficiency. A comparative study

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01797921

Occurrence of Alpha-1-antitrypsin Deficiency in 155 Patients with Alcoholic Liver Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01797921

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01797921

Alpha 1-antitrypsin levels and phenotypes and hepatitis B serology in liver cancer

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01797921

Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency

21 January 2018

1 reference

12 December 2020

Intracellular degradation of the transport-impaired human PiZ alpha 1-antitrypsin variant. Biochemical mapping of the degradative event among compartments of the secretory pathway

1 reference

12 December 2020

Distinctive hepatic cell globules in adult alpha-1-antitrypsin deficiency. A histochemical, immunohistochemical, and ultrastructural study.

1 reference

12 December 2020

Abnormal proteins serve as eukaryotic stress signals and trigger the activation of heat shock genes

1 reference

12 December 2020

Effect of heat shock on protein degradation in mammalian cells: involvement of the ubiquitin system

1 reference

12 December 2020

Prenatal diagnosis of alpha 1 antitrypsin deficiency and estimates of fetal risk for disease

1 reference

12 December 2020

Genetics of alpha 1-antitrypsin deficiency in relation to neonatal liver disease

1 reference

12 December 2020

Transport of secretory and membrane glycoproteins from the rough endoplasmic reticulum to the Golgi. A rate-limiting step in protein maturation and secretion.

1 reference

12 December 2020

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

1 reference

12 December 2020

Hepatocyte Ultrastructural Changes in α1-Antitrypsin Deficiency

1 reference

12 December 2020

Pathogenesis of emphysema. Assessment of basic science concepts through clinical investigation

1 reference

12 December 2020

Liver replacement for alpha1-antitrypsin deficiency

1 reference

12 December 2020

The alpha 1-antitrypsin gene and its deficiency states

1 reference

12 December 2020

Liver transplantation for metabolic disease of the liver.

1 reference

12 December 2020

Evaluation of tamoxifen as a therapy to augment alpha-1-antitrypsin concentrations in Z homozygous alpha-1-antitrypsin-deficient subjects

1 reference

12 December 2020

The alpha 1-antitrypsin gene is expressed in a human intestinal epithelial cell line

1 reference

12 December 2020

BiP expression is not increased by the accumulation of PiZ alpha 1-antitrypsin in the endoplasmic reticulum

1 reference

12 December 2020

In-frame single codon deletion in the Mmalton deficiency allele of alpha 1-antitrypsin

1 reference

12 December 2020

Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder

1 reference

12 December 2020

Expression of the alpha-1-antitrypsin gene in mononuclear phagocytes of normal and alpha-1-antitrypsin-deficient individuals

1 reference

12 December 2020

Effect of administration of a combined estrogen-progestin contraceptive on the level of individual plasma proteins

1 reference

12 December 2020

A multicomponent system that degrades proteins conjugated to ubiquitin. Resolution of factors and evidence for ATP-dependent complex formation.

1 reference

12 December 2020

α-1-antitrypsin phenotypes in hepatocellular carcinoma

1 reference

12 December 2020

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

1 reference

12 December 2020

Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease

1 reference

12 December 2020

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

1 reference

12 December 2020

Repair of the secretion defect in the Z form of alpha 1-antitrypsin by addition of a second mutation

1 reference

12 December 2020

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

1 reference

12 December 2020

The cellular defect in alpha 1-proteinase inhibitor (alpha 1-PI) deficiency is expressed in human monocytes and in Xenopus oocytes injected with human liver mRNA.

1 reference

12 December 2020

Orthotopic liver transplantation for alpha-1-antitrypsin deficiency: an experience in 29 children and ten adults.

1 reference

12 December 2020

The effect of tamoxifen in intermediate alpha 1-antitrypsin deficiency associated with the phenotype PiSZ

1 reference

12 December 2020

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

1 reference

12 December 2020

Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy

1 reference

12 December 2020

The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins

1 reference

12 December 2020

Risk for liver disease in adults with alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Alpha 1 antitrypsinin the livers of patients with emphysema.

1 reference

12 December 2020

Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis

1 reference

12 December 2020

Speculations on the functions of the major heat shock and glucose-regulated proteins

1 reference

12 December 2020

HLA phenotypes and gene polymorphisms in juvenile liver disease associated with α1-antitrypsin deficiency

1 reference

12 December 2020

Change in alpha 1-antitrypsin phenotype after orthotopic liver transplant

1 reference

12 December 2020

A frameshift mutation results in a truncated alpha 1-antitrypsin that is retained within the rough endoplasmic reticulum

1 reference

12 December 2020

Liver transplantation for chronic cholestatic liver disease in adults and children

1 reference

12 December 2020

Accumulation of the insoluble PiZ variant of human alpha 1-antitrypsin within the hepatic endoplasmic reticulum does not elevate the steady-state level of grp78/BiP.

1 reference

12 December 2020

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

1 reference

12 December 2020

Medical problems before and after transplantation

1 reference

12 December 2020

Identification of a serpin-enzyme complex receptor on human hepatoma cells and human monocytes

1 reference

12 December 2020

Liver disease in alpha 1-antitrypsin deficiency. A retrospective analysis of the influence of early breast- vs bottle-feeding

1 reference

12 December 2020

Disruption of the 290-342 salt bridge is not responsible for the secretory defect of the PiZ alpha 1-antitrypsin variant

1 reference

12 December 2020

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

1 reference

12 December 2020

Induction of the stress response in alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Danazol-induced augmentation of serum alpha 1-antitrypsin levels in individuals with marked deficiency of this antiprotease

1 reference

12 December 2020

Evaluation of danazol therapy for patients with PiZZ alpha-1-antitrypsin deficiency

1 reference

12 December 2020

Cirrhosis and malignant hepatoma in alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Human Z alpha 1-antitrypsin accumulates intracellularly and stimulates lysosomal activity when synthesised in the Xenopus oocyte

1 reference

12 December 2020

The alpha 1-antitrypsin gene and its mutations. Clinical consequences and strategies for therapy

1 reference

12 December 2020

Liver disease in infancy: histological features and relationship to alpha-antitrypsin phenotype

1 reference

12 December 2020

Molecular basis of the liver and lung disease associated with the alpha 1-antitrypsin deficiency allele Mmalton

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF01797921

1 reference

16 August 2017

1 reference