In vivo antisense oligonucleotide reduction of NPC1 expression as a novel mouse model for Niemann Pick type C– associated liver disease

Victoria M. Rimkunas, Mark J. Graham, Rosanne M. Crooke, Laura Liscum – 25 April 2008 – Niemann‐Pick type C (NPC) is a fatal autosomal recessive lipidosis that is characterized by lysosomal storage of cholesterol and glycosphingolipids. Patients exhibit prolonged neonatal jaundice, hepatosplenomegaly, and progressive neurodegeneration that generally result in death by the teen years. Most clinical cases are caused by mutations in the NPC1 gene. Current mouse models of NPC are not well suited for studying the liver disease due to the rapidly progressing neurological disease.

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Sanaa Kamal, Michelle Schardell, Samer El Kamary, Mohamed Hashem, Hoda Mansour, Amany Sayed Ibrahim – 25 April 2008

The prevalence and natural history of untreated isolated central perivenulitis in adult allograft livers

Alyssa M. Krasinskas, A. Jake Demetris, John J. Poterucha, Susan C. Abraham – 23 April 2008 – Central perivenulitis (CP) in the allograft liver can be associated with portal‐based acute cellular rejection and autoimmune hepatitis or can occur in isolation (isolated CP). Although several studies have demonstrated the significance of CP, the prevalence and natural history of untreated isolated CP have not been well studied. We examined 100 adult allograft liver recipients who had long‐term follow‐up, had routine protocol biopsies, and received no treatment for isolated CP.

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