Biochemical models of hereditary pancreatitis.

Endocrinology and Metabolism Clinics of North America
Miklós Sahin-Tóth

Abstract

The past decade has witnessed remarkable progress in the genetics of chronic pancreatitis. Despite these accomplishments, the understanding of the molecular mechanisms through which PRSS1 and SPINK1 mutations cause chronic pancreatitis has remained sketchy. Pancreatitis-associated gene mutations are believed to result in uncontrolled trypsin activity in the pancreas. Experimental identification of the disease-relevant functional alterations caused by PRSS1 or SPINK1 mutations proved to be challenging, however, because results of biochemical analyses lent themselves to different interpretations. This article focuses on PRSS1 mutations and summarizes the salient biochemical findings in the context of the mechanistic models that explain the connection between mutations and hereditary pancreatitis.

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Citations

Jan 15, 2008·Langenbeck's Archives of Surgery·Uwe A WittelSurinder K Batra
Oct 6, 2009·The Journal of Biological Chemistry·Eva Kereszturi, Miklós Sahin-Tóth
Jul 31, 2013·Current Opinion in Gastroenterology·Raghuwansh P SahAshok K Saluja
Apr 19, 2011·Pancreas·Maiken T JoergensenMiklós Sahin-Tóth
Sep 1, 2009·Annual Review of Genomics and Human Genetics·Feng ZhangJames R Lupski
May 13, 2010·Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]·Jonas RosendahlMiklós Sahin-Tóth
Dec 30, 2014·BMC Biotechnology·Karin BuettnerThole Zuchner
Aug 30, 2008·Orvosi hetilap·Miklós Sahin-TóthMiklós Tóth
Oct 31, 2006·Nature Genetics·Cédric Le MaréchalClaude Férec
Jan 10, 2014·The Journal of Biological Chemistry·András SzabóMiklós Sahin-Tóth
Aug 1, 2014·The Journal of Biological Chemistry·Raghuwansh P SahAshok K Saluja
Apr 4, 2019·American Journal of Physiology. Gastrointestinal and Liver Physiology·Xianbao ZhanBaoan Ji
Aug 27, 2021·Medicines·Akito TomomuraMineko Tomomura

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Related Papers

Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association
N HowesEuropean Registry of Hereditary Pancreatitis and Pancreatic Cancer (EUROPAC)
The Journal of Biological Chemistry
Zsófia Nemoda, Miklós Sahin-Tóth
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