Non-alcoholic fatty liver disease (NAFLD) is a type of steatosis commonly associated with obesity, dyslipidemia, hypertension, and diabetes. Other diseases such as inherited alpha-1 antitrypsin deficiency (AATD) have also been related to the development of liver steatosis. The primary reasons leading to hepatic lipid deposits can be genetic and epigenetic, and the outcomes range from benign steatosis to liver failure, as well as to extrahepatic diseases. Progressive hepatocellular damage and dysregulated systemic immune responses can affect extrahepatic organs, specifically the heart and lungs. In this review, we discuss the similarities and differences between the molecular pathways of NAFLD and AATD, and the putative value of hepatic organoids as novel models to investigate the physio pathological mechanisms of liver steatosis.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10377048PMC
http://dx.doi.org/10.3390/biomedicines11071961DOI Listing

Publication Analysis

Top Keywords

nafld aatd
8
similarities differences
8
liver steatosis
8
aatd diseases
4
diseases unbalanced
4
unbalanced lipid
4
lipid metabolism
4
metabolism similarities
4
differences non-alcoholic
4
non-alcoholic fatty
4

Similar Publications

Background And Aims: Genetic risk factors are major determinants of chronic liver disease (CLD) progression. Patatin-like phospholipase domain-containing protein 3 (PNPLA3) I148M polymorphism and alpha-1 antitrypsin (AAT) E342K variant, termed PiZ, are major modifiers of metabolic CLD. Both variants are known to affect metabolic CLD through increased endoplasmic reticulum stress, but their combined effect on CLD progression remains largely unknown.

View Article and Find Full Text PDF

Non-alcoholic fatty liver disease (NAFLD) is a type of steatosis commonly associated with obesity, dyslipidemia, hypertension, and diabetes. Other diseases such as inherited alpha-1 antitrypsin deficiency (AATD) have also been related to the development of liver steatosis. The primary reasons leading to hepatic lipid deposits can be genetic and epigenetic, and the outcomes range from benign steatosis to liver failure, as well as to extrahepatic diseases.

View Article and Find Full Text PDF

Background & Aims: Alpha-1 antitrypsin (AAT) deficiency causes/predisposes individuals to advanced chronic liver disease (ACLD). However, the role of the Pi∗Z allele in patients who have already progressed to ACLD is unclear. Thus, we aimed to evaluate the impact of the Pi∗Z allele on the risk of liver transplantation/liver-related death in patients with ACLD, while adjusting for the severity of liver disease at inclusion.

View Article and Find Full Text PDF

[Alpha-1 antitrypsin deficiency: cause and cofactor for liver disease].

Dtsch Med Wochenschr

June 2021

Medizinische Klinik III, Gastroenterologie, Stoffwechselerkrankungen und Intensivmedizin, Uniklinik Aachen, Aachen, Deutschland.

Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder arising due to mutation in alpha1-antitrypsin (AAT). AAT mutations interfere with the AAT production/secretion, cause decreased AAT serum levels and accumulation of AAT in the liver. The excess AAT leads to a proteotoxic liver disease, while the lack of AAT in systemic circulation predisposes to lung injury.

View Article and Find Full Text PDF

Prevalence and clinical characteristics of alpha-1 antitrypsin deficiency in liver explants in a Mexican cohort.

Clin Res Hepatol Gastroenterol

July 2021

Department of Gastroenterology and Hepatology, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Av. Vasco de Quiroga 15, colonia Belisario Domínguez Sección XVI, Tlalpan, 14080 Mexico City, Mexico. Electronic address:

Article Synopsis
  • Alpha-1 antitrypsin deficiency (AATD) is linked to liver disease and was found in around 10% of liver explants from patients, especially those with non-alcoholic steatohepatitis (NASH), suggesting a combined effect of genetic and environmental factors on liver damage.
  • The study analyzed liver samples from 180 patients who underwent liver transplants, with a focus on those with cryptogenic, alcoholic, and NAFLD/NASH cirrhosis, identifying histopathological features indicative of AATD.
  • The results showed AATD in a small percentage (4.5%) of the liver explants, highlighting its rarity in the studied population, but underscoring
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!