Autophagy-enhancing drug carbamazepine diminishes hepatocellular death in fibrinogen storage disease

Journal of Hepatology

Volumn 59, Issue 3, 2013, Pages 626-630

  Puls, Florian ^a   Goldschmidt, Imeke ^a   Bantel, Heike ^a   Agne, Clemens ^a   Bröcker, Verena ^b   Dämmrich, Maximilian ^a   Lehmann, Ulrich ^a   Berrang, Jens ^c   Pfister, Eva Doreen ^a   Kreipe, Hans Heinrich ^a   Baumann, Ulrich ^a  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b ADDENBROOKE S HOSPITAL   (United Kingdom)

^c Klinikzentrum Mitte   (Germany)

Author keywords

Autophagy; Cirrhosis; Elasticity imaging techniques; Fibrinogen; Fibrinogen storage disease

Indexed keywords

ALANINE AMINOTRANSFERASE; CARBAMAZEPINE; CYTOKERATIN 18;

ARTICLE; AUTOPHAGY; CASE REPORT; CELL DEATH; CELL VACUOLE; CHILD; CONTROLLED STUDY; DRUG MECHANISM; ELASTOGRAPHY; ELECTRON MICROSCOPY; FEMALE; FIBRINOGEN DEFECT; FIBRINOGEN STORAGE DISEASE; GENE MUTATION; HUMAN; HYPOFIBRINOGENEMIA; LIVER BIOPSY; LIVER CELL DAMAGE; LIVER FIBROSIS; MALE; PRESCHOOL CHILD; PRIORITY JOURNAL; SCHOOL CHILD; STORAGE DISEASE; TREATMENT RESPONSE;

ALPHA-1-ANTITRYPSIN DEFICIENCY; ALT; ASPARTATE AMINOTRANSFERASE; AST; ATD; AUTOPHAGY; CARBAMAZEPINE; CBZ; CIRRHOSIS; ELASTIC VAN GIESON; ELASTICITY IMAGING TECHNIQUES; ENDOPLASMATIC RETICULUM; ER; EVG; FGC; FIBRINOGEN; FIBRINOGEN GAMMA CHAIN; FIBRINOGEN STORAGE DISEASE; FSD; H&E; HEMATOXYLIN/EOSIN; INR; INTERNATIONAL NORMALIZED RATIO; INTERQUARTILE RANGE; IQR; N.T.; NOT TESTED; OM; ORIGINAL MAGNIFICATION; SERUM ALANINE AMINOTRANSFERASE; TEM; TRANSMISSION ELECTRON MICROSCOPY;

AFIBRINOGENEMIA; ALPHA 1-ANTITRYPSIN DEFICIENCY; AUTOPHAGY; CARBAMAZEPINE; CHILD; CHILD, PRESCHOOL; DNA MUTATIONAL ANALYSIS; ELASTICITY IMAGING TECHNIQUES; ENDOPLASMIC RETICULUM; FEMALE; FIBRINOGEN; HUMANS; LIVER; MALE; PEDIGREE;

EID: 84882946866     PISSN: 01688278     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jhep.2013.05.018     Document Type: Article

References (15)

1. P. de Moerloose, and M. Neerman-Arbez Congenital fibrinogen disorders Semin Thromb Hemost 35 2009 356 366
2. S.O. Brennan, R.L. Davis, K. Conard, A. Savo, and K.N. Furuya Novel fibrinogen mutation gamma314Thr→Pro (fibrinogen AI duPont) associated with hepatic fibrinogen storage disease and hypofibrinogenaemia Liver Int 30 2010 1541 1547
3. B. Ravikumar, S. Sarkar, J.E. Davies, M. Futter, M. Garcia-Arencibia, and Z.W. Green-Thompson Regulation of mammalian autophagy in physiology and pathophysiology Physiol Rev 90 2010 1383 1435
4. T. Hidvegi, M. Ewing, P. Hale, C. Dippold, C. Beckett, and C. Kemp An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis Science 329 2010 229 232
5. S. Kaushal, M. Annamali, K. Blomenkamp, D. Rudnick, D. Halloran, and E.M. Brunt Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model Exp Biol Med (Maywood) 235 2010 700 709
6. D.H. Perlmutter Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates Annu Rev Med 62 2011 333 345
7. K.B. Kruse, A. Dear, E.R. Kaltenbrun, B.E. Crum, P.M. George, and S.O. Brennan Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease Am J Pathol 168 2006 1299 1308
8. D. Joka, K. Wahl, S. Moeller, J. Schlue, B. Vaske, and M.J. Bahr Prospective biopsy-controlled evaluation of cell death biomarkers for prediction of liver fibrosis and nonalcoholic steatohepatitis Hepatology 55 2012 455 464
9. S.O. Brennan, J. Wyatt, D. Medicina, F. Callea, and P.M. George Fibrinogen brescia: hepatic endoplasmic reticulum storage and hypofibrinogenemia because of a gamma284 Gly→Arg mutation Am J Pathol 157 2000 189 196
10. P. Lykavieris, B. Ducot, A. Lachaux, A. Dabadie, P. Broue, and J. Sarles Liver disease associated with ZZ alpha1-antitrypsin deficiency and ursodeoxycholic acid therapy in children J Pediatr Gastroenterol Nutr 47 2008 623 629
11. G. Maggiore, S. Nastasio, and M. Sciveres Long term outcome of liver disease-related fibrinogen Aguadilla storage disease in a child J Pediatr Gastroenterol Nutr 55 2011 699
12. B. Ravikumar, R. Duden, and D.C. Rubinsztein Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy Hum Mol Genet 11 2002 1107 1117
13. B. Ravikumar, Z. Berger, C. Vacher, C.J. O'Kane, and D.C. Rubinsztein Rapamycin pre-treatment protects against apoptosis Hum Mol Genet 15 2006 1209 1216
14. T. Hidvegi, K. Mirnics, P. Hale, M. Ewing, C. Beckett, and D.H. Perlmutter Regulator of G signaling 16 Is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant α1-antitrypsin Z in the classical form of α1-antitrypsin deficiency J Biol Chem 282 2007 27769 27780
15. S.L. Friedman Mechanisms of hepatic fibrogenesis Gastroenterology 134 2008 1655 1669