Wiskott-Aldrich syndrome protein-deficient hematopoietic cells can be efficiently mobilized by granulocyte colony-stimulating factor

Charrier, Sabine; Blundell, Michael P.; Cédrone, Gregory; Louache, Fawzia; Vainchenker, William; Thrasher, Adrian J.; Galy, Anne

doi:10.3324/haematol.2012.077040

Cited by 9 publications

(14 citation statements)

References 39 publications

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“…Although controversial, this data is in line with the observation that WASKO mice have higher numbers of progenitors and granulocytes in spleen and peripheral blood. 23 …”

Section: Resultsmentioning

confidence: 99%

“…WAS patients and animal models exhibit some abnormal hematopoietic parameters. 12 , 23 These abnormalities could be explained by defects in proliferation, migration, and/or apoptosis of the different mature hematopoietic lineages rather than due to a defective hematopoietic development. In fact, most of the data generated from WASKO mice indicate that WASp does not play a major role during hematopoietic development.…”

Section: Discussionmentioning

confidence: 99%

“…However, recent publications showed increased number of hematopoietic progenitors in the peripheral blood and spleen of WASKO mice. 12 , 23 Charrier et al . 23 showed that the total number of progenitors in WASKO mice (taken together CFC from peripheral blood, spleen, and bone marrow) was almost double the total number of progenitors found in WT mice.…”

Section: Discussionmentioning

confidence: 99%

“… 19 , 20 , 21 Similarly, very little is known about the role of WASp in early hematopoiesis with publications with apparent contradictory results. 6 , 22 , 23 …”

Section: Introductionmentioning

confidence: 99%

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Absence of WASp Enhances Hematopoietic and Megakaryocytic Differentiation in a Human Embryonic Stem Cell Model

et al. 2016

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The Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency caused by mutations in the WAS gene and characterized by severe thrombocytopenia. Although the role of WASp in terminally differentiated lymphocytes and myeloid cells is well characterized, its role in early hematopoietic differentiation and in platelets (Plts) biology is poorly understood. In the present manuscript, we have used zinc finger nucleases targeted to the WAS locus for the development of two isogenic WAS knockout (WASKO) human embryonic stem cell lines (hESCs). Upon hematopoietic differentiation, hESCs-WASKO generated increased ratios of CD34+CD45+ progenitors with altered responses to stem cell factor compared to hESCs-WT. When differentiated toward the megakaryocytic linage, hESCs-WASKO produced increased numbers of CD34+CD41+ progenitors, megakaryocytes (MKs), and Plts. hESCs-WASKO-derived MKs and Plts showed altered phenotype as well as defective responses to agonist, mimicking WAS patients MKs and Plts defects. Interestingly, the defects were more evident in WASp-deficient MKs than in WASp-deficient Plts. Importantly, ectopic WAS expression using lentiviral vectors restored normal Plts development and MKs responses. These data validate the AND-1_WASKO cell lines as a human cellular model for basic research and for preclinical studies for WAS.

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“…Although controversial, this data is in line with the observation that WASKO mice have higher numbers of progenitors and granulocytes in spleen and peripheral blood. 23 …”

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“… 19 , 20 , 21 Similarly, very little is known about the role of WASp in early hematopoiesis with publications with apparent contradictory results. 6 , 22 , 23 …”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Absence of WASp Enhances Hematopoietic and Megakaryocytic Differentiation in a Human Embryonic Stem Cell Model

et al. 2016

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“…The impact of WASP gene deficiency on mobilization of hematopoietic progenitor/stem cells in circulation remains unknown, but previous research has indicated a correlation in the context of autologous gene therapy of Wiskott-Aldrich syndrome. In one study using a murine WASP-knockout model, Charrier et al found the occurrence of B-cell lymphopenia, marked neutrophilia, increased counts of circulating hematopoietic progenitor cells, and splenomegaly in WASP-knockout mice in the steady state, all of which were presumably caused by retention of hematopoietic progenitor cells due to high levels of splenic CXCL12 ( 11 ).…”

Section: Pathogenesis Of Hypersplenismmentioning

confidence: 99%

Hypersplenism: History and current status

Lau

et al. 2016

Experimental and Therapeutic Medicine

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Hypersplenism is a common disorder characterized by an enlarged spleen which causes rapid and premature destruction of blood cells. This review summarizes the history of hypersplenism, discuss its classification and pathogenesis, and examines its diagnosis and treatment options. We performed a comprehensive literature search using PubMed, Web of Knowledge and the China National Knowledge Infrastructure (CNKI) database, reviewed hypersplenism-related articles and summarized the major findings. According to its etiological causes, hypersplenism is characterized by splenomegaly and peripheral cytopenias. It can be classified into three categories: i) primary hypersplenism; ii) secondary hypersplenism; and iii) occult hypersplenism. A number of mechanisms causing hypersplenism have been identified, and mainly involve retention in the spleen, phagocytosis, and autoimmunity. Treatment options for hypersplenism include etiological treatment, non-surgical treatment, total splenectomy and liver transplantation. In any case, treatment should be individualized for each patient.

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A single-cell atlas of immunocytes in the spleen of a mouse model of Wiskott-Aldrich syndrome

Liang,

Peng,

Luo

et al. 2023

Cellular Immunology

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Wiskott-Aldrich syndrome protein-deficient hematopoietic cells can be efficiently mobilized by granulocyte colony-stimulating factor

Cited by 9 publications

References 39 publications

Absence of WASp Enhances Hematopoietic and Megakaryocytic Differentiation in a Human Embryonic Stem Cell Model

Absence of WASp Enhances Hematopoietic and Megakaryocytic Differentiation in a Human Embryonic Stem Cell Model

Hypersplenism: History and current status

A single-cell atlas of immunocytes in the spleen of a mouse model of Wiskott-Aldrich syndrome

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