scholarly journals Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency

2012 ◽  
Vol 237 (10) ◽  
pp. 1163-1172 ◽  
Author(s):  
Nancy Y Marcus ◽  
Keith Blomenkamp ◽  
Muneeb Ahmad ◽  
Jeffrey H Teckman
Keyword(s):  
Oxidative Stress ◽  
Liver Damage ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Indomethacin increases liver damage in a murine model of liver injury from alpha-1-antitrypsin deficiency

Hepatology ◽  
2006 ◽  
Vol 44 (4) ◽  
pp. 976-982 ◽  
Author(s):  
David A. Rudnick ◽  
Olga Shikapwashya ◽  
Keith Blomenkamp ◽  
Jeffrey H. Teckman
Keyword(s):  
Liver Injury ◽  
Liver Damage ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Oxidative Stress and Polymorphism of Xenobiotic-Metabolizing Enzymes in Two Patients with Severe Alpha-1-Antitrypsin Deficiency

2016 ◽  
Vol 54 (5) ◽  
pp. 746-752 ◽  
Author(s):  
Aleksandra Topic ◽  
Ljudmila Nagorni-Obradovic ◽  
Djordje Francuski ◽  
Mila Ljujic ◽  
Zivka Malic ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Metabolizing Enzymes ◽  
Xenobiotic Metabolizing Enzymes ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Pathophysiological aspects of liver damage in children with alpha-1-antitrypsin deficiency

2020 ◽  
Vol 65 (1) ◽  
pp. 11-21
Author(s):  
G. V. Volynets ◽  
A. V. Nikitin
Keyword(s):  
Liver Disease ◽  
Liver Damage ◽  
Autosomal Recessive ◽  
Neonatal Period ◽  
Autosomal Recessive Disease ◽  
Clinical Manifestations ◽  
Homozygous Mutation ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Alpha-1-antitrypsin deficiency is an autosomal recessive disease characterized by both liver damage and lung disease in children and adults because of a decrease in the serum protein content due to the mutations in the PI (proteinase inhibitor) gene. The majority of liver diseases are associated with a homozygous mutation of the Z allele. There are many variations of clinical manifestations of the liver disease in children with the PI*ZZ genotype.  In the neonatal period, liver disease is usually cholestatic; and it is accompanied by a prolonged cholestatic jaundice, skin itching, which can be determined only later (after 6 months), decreased appetite and bad weight gain, hepato- and splenomegaly. The article describes the pathophysiology of liver damage in children with alpha-1-antitrypsin deficiency. The authors provide their recommendations for the management of children with suspected and confirmed alpha-1-antitrypsin deficiency.

scholarly journals Oxidative Stress and Endoplasmic Reticulum Stress in Rare Respiratory Diseases

2021 ◽  
Vol 10 (6) ◽  
pp. 1268
Author(s):  
María Magallón ◽  
Ana Esther Carrión ◽  
Lucía Bañuls ◽  
Daniel Pellicer ◽  
Silvia Castillo ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Cystic Fibrosis ◽  
Endoplasmic Reticulum ◽  
Primary Ciliary Dyskinesia ◽  
Respiratory Diseases ◽  
Therapeutic Agents ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Ciliary Dyskinesia

Several studies have shown that some rare respiratory diseases, such as alpha-1 antitrypsin deficiency (AATD), idiopathic pulmonary fibrosis (IPF), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD) present oxidative stress (OS) and endoplasmic reticulum (ER) stress. Their involvement in these pathologies and the use of antioxidants as therapeutic agents to minimize the effects of OS are discussed in this review.

LSC Abstract – Increased oxidative stress leads to telomere shortening in children with alpha-1 antitrypsin deficiency

2016 ◽  
Author(s):  
Sara Pastor ◽  
Amparo Escribano ◽  
Silvia Castillo ◽  
Ana Reula ◽  
María Mercedes Navarro-García ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Telomere Shortening ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

LSC Abstract – Increased oxidative stress leads to telomere shortening in children with alpha-1 antitrypsin deficiency

2016 ◽  
Author(s):  
Sara Pastor ◽  
Amparo Escribano ◽  
Silvia Castillo ◽  
Ana Reula ◽  
María Mercedes Navarro-García ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Telomere Shortening ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Characteristics of hepatocellular carcinoma in a murine model of alpha-1-antitrypsin deficiency

2010 ◽  
Vol 40 (6) ◽  
pp. 641-653 ◽  
Author(s):  
Nancy Y. Marcus ◽  
Elizabeth M. Brunt ◽  
Keith Blomenkamp ◽  
Faiza Ali ◽  
David A. Rudnick ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Murine Model ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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