Acute hepatic porphyrias: Recommendations for evaluation and long‐term management

Manisha Balwani, Bruce Wang, Karl E. Anderson, Joseph R. Bloomer, D. Montgomery Bissell, Herbert L. Bonkovsky, John D. Phillips, Robert J. Desnick, for the Porphyrias Consortium of the Rare Diseases Clinical Research Network – 12 June 2017 – The acute hepatic porphyrias are a group of four inherited disorders, each resulting from a deficiency in the activity of a specific enzyme in the heme biosynthetic pathway. These disorders present clinically with acute neurovisceral symptoms which may be sporadic or recurrent and, when severe, can be life‐threatening.

Calcium‐binding protein 39 promotes hepatocellular carcinoma growth and metastasis by activating extracellular signal‐regulated kinase signaling pathway

Lingxi Jiang, Qian Yan, Shuo Fang, Ming Liu, Yan Li, Yun‐Fei Yuan, Yan Li, Yinghui Zhu, Jiali Qi, Xiaodong Yang, Dora Lai Wan Kwong, Xin‐Yuan Guan – 12 June 2017 – Calcium‐binding protein (CAB39) is a key regulator of a group of sterile 20 kinases. Here, we report that CAB39 was frequently up‐regulated in hepatocellular carcinoma (HCC), which was significantly associated with tumor metastasis (P = 0.000), poorer disease‐free survival rate (P = 0.027), and poor prognosis (P = 0.000).

Acute hepatic porphyrias: Recommendations for evaluation and long‐term management

Manisha Balwani, Bruce Wang, Karl E. Anderson, Joseph R. Bloomer, D. Montgomery Bissell, Herbert L. Bonkovsky, John D. Phillips, Robert J. Desnick, for the Porphyrias Consortium of the Rare Diseases Clinical Research Network – 12 June 2017 – The acute hepatic porphyrias are a group of four inherited disorders, each resulting from a deficiency in the activity of a specific enzyme in the heme biosynthetic pathway. These disorders present clinically with acute neurovisceral symptoms which may be sporadic or recurrent and, when severe, can be life‐threatening.

Hepatic lipophagy: New insights into autophagic catabolism of lipid droplets in the liver

Ryan J. Schulze, Kristina Drižytė, Carol A. Casey, Mark A. McNiven – 9 June 2017 – The liver is a central fat‐storage organ, making it especially susceptible to steatosis as well as subsequent inflammation and cirrhosis. The mechanisms by which the liver mobilizes stored lipid for energy production, however, remain incompletely defined. The catabolic process of autophagy, a well‐known process of bulk cytoplasmic recycling and cellular self‐regeneration, is a central regulator of lipid metabolism in the liver.

Serum microcystin levels positively linked with risk of hepatocellular carcinoma: A case‐control study in southwest China

Chuanfen Zheng, Hui Zeng, Hui Lin, Jia Wang, Xiaobin Feng, Zhiqun Qiu, Ji‐an Chen, Jiaohua Luo, Yang Luo, Yujing Huang, Lingqiao Wang, Wenyi Liu, Yao Tan, Anwei Xu, Yuan Yao, Weiqun Shu – 9 June 2017 – Microcystins have been reported to be carcinogenic by animal and cell experimentation, but there are no data on the linkage between serum microcystins and hepatocellular carcinoma (HCC) risk in humans.

Bone morphogenetic protein signaling governs biliary‐driven liver regeneration in zebrafish through tbx2b and id2a

Tae‐Young Choi, Mehwish Khaliq, Shinya Tsurusaki, Nikolay Ninov, Didier Y.R. Stainier, Minoru Tanaka, Donghun Shin – 9 June 2017 – Upon mild liver injury, new hepatocytes originate from preexisting hepatocytes. However, if hepatocyte proliferation is impaired, a manifestation of severe liver injury, biliary epithelial cells (BECs) contribute to new hepatocytes through BEC dedifferentiation into liver progenitor cells (LPCs), also termed oval cells or hepatoblast‐like cells (HB‐LCs), and subsequent differentiation into hepatocytes.

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