2015
DOI: 10.1084/jem.20141286
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Abstract: Rissone et al. demonstrate that adenylate kinase AK2, an enzyme mutated in reticular dysgenesis (RD) in humans, prevents oxidative stress during hematopoiesis. Using a zebrafish model, as well as induced pluripotent stem cells derived from an RD patient, they find that AK2 deficiency affects hematopoietic stem and progenitor development with increased oxidative stress. Antioxidant treatment rescues the hematopoietic phenotypes.

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Cited by 51 publications
(51 citation statements)
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“…the morphants exhibited defects in leucocyte development with absent T cells in the thymus anlage (Pannicke et al, 2009). In a refinement of the zebrafish studies, ak2 mutant lines showed a profound impairment of lymphoid as well as myeloid cell development (Rissone et al, 2015). Additionally, a markedly reduced expression of the erythroid marker hbae1.1 was noted, supporting the idea that erythropoiesis defects are intrinsic to Ak2 deficiency in this model.…”
Section: Pathophysiology Of Ak2 Deficiency In Cellular and Animal Modelsmentioning
confidence: 61%
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“…the morphants exhibited defects in leucocyte development with absent T cells in the thymus anlage (Pannicke et al, 2009). In a refinement of the zebrafish studies, ak2 mutant lines showed a profound impairment of lymphoid as well as myeloid cell development (Rissone et al, 2015). Additionally, a markedly reduced expression of the erythroid marker hbae1.1 was noted, supporting the idea that erythropoiesis defects are intrinsic to Ak2 deficiency in this model.…”
Section: Pathophysiology Of Ak2 Deficiency In Cellular and Animal Modelsmentioning
confidence: 61%
“…The observation of a large fraction of anaemic RD patients at birth complements these observations (Hoenig et al, 2017). Furthermore, Rissone et al (2015) showed that increased oxidative stress and reactive oxygen species (ROS) production results in cell death and apoptosis and that Ak2 deficiency causes a progressive reduction of haematopoietic stem and progenitor cells by this mechanism. These developmental blocks in the Ak2-deficient zebrafish model could be reversed by antioxidative treatment.…”
Section: Pathophysiology Of Ak2 Deficiency In Cellular and Animal Modelsmentioning
confidence: 67%
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“…Rissone et al 29 used patient-derived induced pluripotent stem cells and a zebrafish model to demonstrate that deficiency of adenylate kinase 2 (AK2), responsible for reticular dysgenesis, affects hematopoietic stem and progenitor cell development and increases oxidative stress and apoptosis of myeloid progenitor cells. These abnormalities were rescued by addition of anti-oxidant drugs.…”
Section: Immunopathogenesis Of Primary Immunodeficienciesmentioning
confidence: 99%