Results 101 to 110 of about 256,046 (232)

Reconstruction of the mouse extrahepatic biliary tree using primary human extrahepatic cholangiocyte organoids [PDF]

, 2017
Treatment of common bile duct disorders such as biliary atresia or ischaemic strictures is limited to liver transplantation or hepatojejunostomy due to the lack of suitable tissue for surgical reconstruction.
Alexander, Graeme J., Bargehr, Johannes, Berntsen, Natalie Lie, Bertero, Alessandro, Brown, Stephanie, Casey, John, Davies, Susan E., de Brito, Miguel Cardoso, Gelson, William T.H., Georgakopoulos, Nikitas, Gieseck, Richard L., Godfrey, Edmund M., Gómez-Vázquez, María J., Hannan, Nicholas R.F., Jensen, Kim B., Justin, Alexander W., Karlsen, Tom H., Madrigal, Pedro, Markaki, Athina E., Materek, Paulina M., Melum, Espen, Ortmann, Daniel, Pawlowski, Matthias, Pedersen, Marianne T., Pirmadjid, Negar, Rimland, Casey A., Ross, Alexander, Saeb-Parsy, Kourosh, Sampaziotis, Fotios, Sawiak, Stephen, Shu, Wenmiao, Simonic, Ingrid, Sinha, Sanjay, Skeldon, Gregor M., Snijders, Kirsten, Tysoe, Olivia C., Upponi, Sara S., Valestrand, Laura, Vallier, Ludovic, Wynn, Thomas A., Yiangou, Loukia, Zilbauer, Matthias, Zonneveld, Mariëlle C.F.   +42 more
core   +7 more sources

Transcriptomic study in explanted liver from a patient with acute intermittent porphyria

JIMD Reports, 2023
Acute intermittent porphyria (AIP) is a rare disease caused by a deficiency of hydroxymethylbilane synthase (HMBS), the third enzyme of the heme‐synthesis pathway. Decreased enzymatic activity in the liver induces an overproduction of heme‐precursors and
Jordi To‐Figueras, Esther Titos, Paula Aguilera, Alba Díaz, Javier Muñoz‐Luque, Irene Madrigal, Celia Badenas, Mercè Torra, Constantino Fondevila, Jordi Colmenero   +9 more
doaj   +1 more source

Carrier detection and phenotypic expression in a family with hereditary coproporphyria [PDF]

, 2007
University of Technology, Sydney. Faculty of Science.Introduction: Hereditary coproporphyria (HCP) is an autosomal dominant disorder that results from defects in the enzyme coproporphyrinogen oxidase (CPOX).
Al Hafid, N
core  

New mutations of the hydroxymethylbilane synthase gene in German patients with acute intermittent porphyria

Molecular and Cellular Probes, 1999
Acute intermittent porphyria (AIP) is a low-penetrant, autosomal dominant disorder caused by decreased activity of hydroxymethylbilane synthase (HMBS; MIM 176 000), the third enzyme in the heme biosynthetic pathway. We report the first molecular analysis of HMBS gene mutations in classical AIP patients of German origin.
U, Gross, H, Puy, M, Doss, A M, Robreau, Y, Nordmann, M O, Doss, J C, Deybach   +6 more
openaire   +2 more sources

Increased Mitochondrial Superoxide Level Is Partially Associated With Vemurafenib‐Induced Renal Tubular Toxicity

Basic &Clinical Pharmacology &Toxicology, Volume 136, Issue 4, April 2025.
Vemurafenib (VEM) induces renal tubular toxicity through mitochondrial dysfunction and lysosomal abnormalities. Superoxide production is a key factor, and mitochondrial antioxidant XJB‐5‐131 partially alleviated this toxicity. Autophagy impairment is also suggested.
Akimasa Sanagawa, Hiroshi Takase
wiley   +1 more source

Reference gene selection for the shell gland of laying hens in response to time-points of eggshell formation and nicarbazin. [PDF]

PLoS ONE, 2017
Ten reference genes were investigated for normalization of gene expression data in the shell gland of laying hens. Analyses performed with geNorm revealed that hypoxanthine phosphoribosyltransferase 1 (HPRT1) and hydroxymethylbilane synthase (HMBS) were ...
Sami Samiullah, Juliet Roberts, Shu-Biao Wu   +2 more
doaj   +1 more source

Continued 26S proteasome dysfunction in mouse brain cortical neurons impairs autophagy and the Keap1-Nrf2 oxidative defence pathway [PDF]

, 2017
The ubiquitin–proteasome system (UPS) and macroautophagy (autophagy) are central to normal proteostasis and interdependent in that autophagy is known to compensate for the UPS to alleviate ensuing proteotoxic stress that impairs cell function.
A Ciechanover, A Khaminets, A Kobayashi, A Ordureau, A Orsi, A Shevchenko, A Stolz, A Tonoki, A Yerlikaya, AM Pickrell, AM Pickrell, BE Riley, CC Tan, D Narendra, D Vilchez, DJ Klionsky, FH Sterky, G Matsumoto, G Matsumoto, HK Bryan, I Tanida, International Genomics of Alzheimer's Disease C, J Cox, J Elkharaz, J Fullgrabe, J Lim, J Lowe, J Zhao, J Zirin, K Taguchi, K Tanaka, K Yamano, L Bedford, LJ Kraft, M Jin, M Kobayashi, M Komatsu, M Zhu, NC Chan, P Xia, P Xia, Q Ding, RC Russell, RW Button, S Kageyama, S Svenning, SA Sarraf, SH Ro, SM Paine, SR Yoshii, T Hara, V Nikoletopoulou, V Rogov, VI Korolchuk, W Filipowicz, WK Wu, Y Ichimura, Y Tashiro, YC Wong, Z Khalkhali-Ellis   +59 more
core   +3 more sources

Liver transplantation and primary liver cancer in porphyria

Liver International, Volume 45, Issue 3, March 2025.
Abstract The porphyrias are a heterogeneous group of metabolic disorders that result from defects in heme synthesis. The metabolic defects are present in all cells, but symptoms are mainly cutaneous or related to neuropathy. The porphyrias are highly relevant to hepatologists since patients can present with symptoms and complications that require liver
Mattias Lissing, Bruce Wang, Staffan Wahlin   +2 more
wiley   +1 more source

No evidence for sylvatic cycles of chikungunya, dengue and Zika viruses in African green monkeys (Chlorocebus aethiops sabaeus) on St. Kitts, West Indies

Parasites & Vectors, 2020
Background Dengue, chikungunya and Zika viruses (DENV, CHIKV and ZIKV) are transmitted in sylvatic transmission cycles between non-human primates and forest (sylvan) mosquitoes in Africa and Asia.
Matthew John Valentine, Brenda Ciraola, Matthew Thomas Aliota, Michel Vandenplas, Silvia Marchi, Bernard Tenebray, Isabelle Leparc-Goffart, Christa Ann Gallagher, Amy Beierschmitt, Tatiana Corey, Kerry McAuliffe Dore, Xavier de Lamballerie, Chengming Wang, Courtney Cuin Murdock, Patrick John Kelly   +14 more
doaj   +1 more source

Zinc signaling controls astrocyte‐dependent synapse modulation via the PAF receptor pathway

Journal of Neurochemistry, Volume 169, Issue 2, February 2025.
Low zinc bioavailability triggers inflammatory signaling through chronic activation of astrocytes via the PAFR. Active astrocytes release ROS, metabolites, and cytokines that affect synapse formation in developing neurons. Adequate zinc status prevents astrocyte (over)activation. PAFR, platelet activation factor receptor.
Janelle E. Stanton, Sakshi Hans, Ioannis Zabetakis, Andreas M. Grabrucker   +3 more
wiley   +1 more source