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LPD Associated with Epstein–Barr Virus Infection
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
EBV-driven LPD or LPD associated with EBV infection are rare diseases, with estimated incidences of 1 in 1–5 million people. To date, <1000 cases of EBV-driven LPD have been reported, including XLP1 (>100 cases), XLP2 (>100 cases), CD27 deficiency (18 cases), ITK deficiency (13 cases), CTPS1 deficiency (12 cases), MAGT1 deficiency (11 cases), ZAP70 deficiency (10 cases), and coronin 1A deficiency (9 cases) (Table 75.1). However, EBV-driven LPD may be underdiagnosed due to its severity and often rapidly fatal initial presentation, variable expression, and clinical overlap with other immunologic disorders, in addition to the absence of functional diagnostic assays [1].
Lessons from transmissible cancers for immunotherapy and transplant
Published in Immunological Medicine, 2022
Rafael Cardoso Maciel Costa Silva, Carolina Panis, Bruno Ricardo Barreto Pires
Coronin 1 belongs to an evolutionary-conserved family of proteins, involved in cytoskeleton dynamics in yeast [186] and T cell survival and signaling regulation in mammals [187]. Expressed in different leukocytes, coronin 1 has been described as an essential and specific target for transplants, without restraining immune response against infectious agents [185,188]. Coronin 1 deficiency leads to reduced phosphodiesterase 4 (PDE4) activity, resulting in increased cyclic AMP (cAMP) levels in T cells. Higher cAMP levels in T conventional cells renders them ineffective to drive immune response against allogeneic antigens, but not microbial-derived ones. Thus, the axis coronin 1-PDE4-cAMP is an intriguing molecular target to be evaluated as an activator of T cell-mediated anti-tumor immunity. PDE4 activators or protein kinase A inhibitors are already described as pharmacological modulators [185,189] that can be used to decrease cAMP or modulate cAMP downstream signaling, respectively, in T cells and possibly improve adaptive immunity against neoantigens (similar to allogeneic ones).
Biochemical and immunocytochemical characterization of coronins in platelets
Published in Platelets, 2020
David R. J. Riley, Jawad S. Khalil, Khalid M. Naseem, Francisco Rivero
Coronins constitute a family of conserved regulators of the actin cytoskeleton turnover. The defining architectural element of this family is the WD40 repeat that folds in a β-propeller structure and characteristically participates in protein–protein interactions [6]. The β-propeller is flanked by short highly conserved extensions. The C-terminal extension is followed by a variable unique region and a coiled-coil domain, and the latter involved in oligomerization [7,8]. Mammals express seven coronins that have been grouped into three classes [9,10]. Among class I coronins (Coro1, 2, 3 and 6), Coro1 is the most widely studied for its role in coordinating actin dynamics through modulation of Arp2/3 complex and cofilin function [11]. Coro1 also plays less well-understood roles in NADPH oxidase complex regulation, calcium release, vesicle trafficking and apoptosis [12–15]. Class I coronins localize at the leading edge of migrating cells and to phagosomes in neutrophils [7,15,16]. Class II coronins (Coro4 and 5) are involved in focal adhesion turnover, reorganization of the cytoskeleton and cell migration [17,18]. The class III coronin (Coro7) has an unusual structure, as it consists of two coronin blocks in tandem and lacks a coiled-coil region. This atypical coronin plays a role in Golgi morphology maintenance and does not appear to participate in actin-related processes [19].
Regulation of differentiation of MEG01 to megakaryocytes and platelet-like particles by Valproic acid through Notch3 mediated actin polymerization
Published in Platelets, 2019
Ankita Dhenge, Rutuja Kuhikar, Vaijayanti Kale, Lalita Limaye
Based on our data and these reports, we speculate that Valproic acid upregulates 14–3-3 zeta that controls PLT activation via Rac/Cdc42 GTPase. Coronin 1 A negatively regulates F-actin polymerization. In other words, downregulation of Coronin 1A supports proplatelet formation from MKs and is enhanced upon VPA-mediated differentiation.