Abstract While ATF6α plays a central role in the endoplasmic reticulum (ER) stress response, function of its paralogue ATF6β remains elusive, especially nervous system (CNS). Here, we demonstrate that is highly expressed hippocampus brain, and specifically regulates expression calreticulin (CRT), molecular chaperone ER with high Ca 2+ -binding capacity. CRT was reduced to ~ 50% CNS Atf6b −/− mi...

Chronic neutrophilic leukaemia (CNL) is a rare type of myeloproliferative neoplasm (MPN) characterised by sustained leucocytosis (≥25×10(9)/L) with neoplastic proliferation of neutrophilic granulocytes in blood and bone marrow. In contrast to chronic myeloid leukaemia, the disease primarily involves neutrophilic lineage with persistent proliferation of mature forms of neutrophils. No consistent...

This article tries to answer the following questions: why we are in need of a revision of the prior diagnostic criteria for Philadelphia -ve MPNs, what is new in 2016 multiparameter WHO classification for Philadelphia-ve MPNs and how a single JAK2V617F mutation can lead to three different MPNs. In addition it discusses the genetic basis and molecular pathophysiology of Philadelphia-ve classical...

Myeloproliferative neoplasms (MPNs) are the most common underlying prothrombotic disorder found in patients with splanchnic vein thrombosis (SVT). Clinical risk factors for MPN-associated SVTs include younger age, female sex, concomitant hypercoagulable disorders, and the JAK2 V617F mutation. These risk factors are distinct from those associated with arterial or deep venous thrombosis (DVT) in ...