Matches in SemOpenAlex for { <https://semopenalex.org/work/W3208121446> ?p ?o ?g. }
- W3208121446 endingPage "79" @default.
- W3208121446 startingPage "65" @default.
- W3208121446 abstract "Alpha-1 antitrypsin (AAT), encoded by the SERPINA1 gene, is a protein mainly produced and secreted by hepatocytes. Some specific mutations affecting SERPINA1 may cause accumulation of misfolded AAT in the endoplasmic reticulum of the hepatocytes leading to AAT deficiency (AATD). Z-AAT is the most severe and common deficient variant. This mutant is not only retained in the endoplasmic reticulum but accumulates as an aggregate that triggers a cascade of intracellular signalling pathways inducing hepatocyte injury and death. Nevertheless, among all the homozygous ZZ patients only 15% develop liver injury, with a wide-range of disease severities ranging from hepatic fibrosis to cirrhosis or even hepatocellular carcinoma. Due to the lack of knowledge surrounding modifiers associated with Z-AAT-mediated hepatocyte toxicity, it is impossible to screen for AATD patients at risk of liver damage and to develop accurate therapeutic strategies. This review aims to give an overview and update our knowledge of AATD associated with liver disease and discusses possible new therapeutic strategies." @default.
- W3208121446 created "2021-11-08" @default.
- W3208121446 creator A5004025888 @default.
- W3208121446 creator A5062191903 @default.
- W3208121446 creator A5068033382 @default.
- W3208121446 creator A5081268394 @default.
- W3208121446 creator A5091792031 @default.
- W3208121446 date "2019-05-28" @default.
- W3208121446 modified "2023-10-03" @default.
- W3208121446 title "Overview of Alpha-1 Antitrypsin Deficiency-Mediated Liver Disease" @default.
- W3208121446 cites W1484734537 @default.
- W3208121446 cites W1555987236 @default.
- W3208121446 cites W1900633721 @default.
- W3208121446 cites W1963818067 @default.
- W3208121446 cites W1964727784 @default.
- W3208121446 cites W1973448864 @default.
- W3208121446 cites W1975200363 @default.
- W3208121446 cites W1977373454 @default.
- W3208121446 cites W1978907644 @default.
- W3208121446 cites W1984582197 @default.
- W3208121446 cites W1985494921 @default.
- W3208121446 cites W1993997684 @default.
- W3208121446 cites W1998289395 @default.
- W3208121446 cites W2000335482 @default.
- W3208121446 cites W2003400191 @default.
- W3208121446 cites W2008958864 @default.
- W3208121446 cites W2009269185 @default.
- W3208121446 cites W2015811490 @default.
- W3208121446 cites W2019739887 @default.
- W3208121446 cites W2024125817 @default.
- W3208121446 cites W2027140570 @default.
- W3208121446 cites W2029314935 @default.
- W3208121446 cites W2032492315 @default.
- W3208121446 cites W2040562670 @default.
- W3208121446 cites W2049022764 @default.
- W3208121446 cites W2054159211 @default.
- W3208121446 cites W2063835280 @default.
- W3208121446 cites W2064028385 @default.
- W3208121446 cites W2064810365 @default.
- W3208121446 cites W2069484087 @default.
- W3208121446 cites W2073346335 @default.
- W3208121446 cites W2074863064 @default.
- W3208121446 cites W2077499130 @default.
- W3208121446 cites W2080363121 @default.
- W3208121446 cites W2083120544 @default.
- W3208121446 cites W2094499644 @default.
- W3208121446 cites W2095374572 @default.
- W3208121446 cites W2099937253 @default.
- W3208121446 cites W2100985059 @default.
- W3208121446 cites W2104564301 @default.
- W3208121446 cites W2108178698 @default.
- W3208121446 cites W2117383725 @default.
- W3208121446 cites W2117818786 @default.
- W3208121446 cites W2117894506 @default.
- W3208121446 cites W2118611462 @default.
- W3208121446 cites W2136278161 @default.
- W3208121446 cites W2136866051 @default.
- W3208121446 cites W2158442258 @default.
- W3208121446 cites W2165156191 @default.
- W3208121446 cites W2165209066 @default.
- W3208121446 cites W2165741321 @default.
- W3208121446 cites W2233119733 @default.
- W3208121446 cites W2271599132 @default.
- W3208121446 cites W2290980437 @default.
- W3208121446 cites W2325223078 @default.
- W3208121446 cites W2334275983 @default.
- W3208121446 cites W2512326554 @default.
- W3208121446 cites W2571086437 @default.
- W3208121446 cites W2625343909 @default.
- W3208121446 cites W2650954899 @default.
- W3208121446 cites W2753205476 @default.
- W3208121446 cites W2774922482 @default.
- W3208121446 cites W2777113886 @default.
- W3208121446 cites W2791744212 @default.
- W3208121446 cites W2793779340 @default.
- W3208121446 cites W2800936887 @default.
- W3208121446 cites W2806638042 @default.
- W3208121446 cites W2806754254 @default.
- W3208121446 cites W2888207000 @default.
- W3208121446 cites W2907576011 @default.
- W3208121446 cites W2912115654 @default.
- W3208121446 cites W4210641080 @default.
- W3208121446 cites W4229980143 @default.
- W3208121446 cites W4236661420 @default.
- W3208121446 cites W4246051404 @default.
- W3208121446 cites W4248015635 @default.
- W3208121446 cites W4252288828 @default.
- W3208121446 cites W4296231508 @default.
- W3208121446 doi "https://doi.org/10.33590/emjhepatol/10314658" @default.
- W3208121446 hasPublicationYear "2019" @default.
- W3208121446 type Work @default.
- W3208121446 sameAs 3208121446 @default.
- W3208121446 citedByCount "2" @default.
- W3208121446 countsByYear W32081214462021 @default.
- W3208121446 countsByYear W32081214462022 @default.
- W3208121446 crossrefType "journal-article" @default.
- W3208121446 hasAuthorship W3208121446A5004025888 @default.
- W3208121446 hasAuthorship W3208121446A5062191903 @default.