Paroxysmal nocturnal hemoglobinuria without GPI-anchor deficiency

Brodsky, Robert A.

doi:10.1172/jci131647

Cited by 6 publications

(3 citation statements)

References 19 publications

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“…Erythrocytes in paroxysmal nocturnal hemoglobinuria are deficient in glycosyl-phosphatidylinositol anchor proteins, such as CD55 (decay-accelerating factor) and CD59 (membrane attack complex inhibition factor), resulting in complement-mediated hemolysis. 26 By flow cytometry we found that neither CD55 nor CD59a were reduced ( Figure 3 E). Ham test results confirmed that Gata1s erythrocytes were not more sensitive to complement-mediated lysis ( supplemental Figure 4 A-B).…”

Section: Resultsmentioning

confidence: 89%

Gata1s mutant mice display persistent defects in the erythroid lineage

et al. 2023

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GATA1 mutations that result in loss of the N-terminal 83 amino acids are a feature of myeloid leukemia in children with Down syndrome (ML-DS), rare familial cases of dyserythropoietic anemia, and a subset of cases of Diamond-Blackfan Anemia (DBA). The Gata1s mouse model, which expresses only the short GATA1 isoform that begins at methionine 84, has been shown to have a defect in hematopoiesis, specially impaired erythropoiesis with expanded megakaryopoiesis, during gestation. However, the mice were reported to not show any post-natal phenotype. Here we demonstrate that Gata1s mutant mice display macrocytic anemia and features of aberrant megakaryopoiesis throughout life, culminating in profound splenomegaly and bone marrow fibrosis. These data support the use of this animal model for studies of GATA1 deficiencies.

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Section: Resultsmentioning

confidence: 89%

Gata1s mutant mice display persistent defects in the erythroid lineage

et al. 2023

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“…16 The loss of GPI-Aps are the cause of paroxysmal nocturnal hemoglobinuria. 17 GPI-anchored proteins are also in a key position to influence signal transduction in malignancies. For example, Zhang et al confirmed that GPI-anchored CD109 downregulates TGF-β1 signaling and enhances EGF signaling in glioblastoma cells.…”

Section: Glycosylation and Its Importance In Normal Cellular Functionmentioning

confidence: 99%

“…Disruptions in GPI‐anchored proteins (GPI‐Aps) are reported to be involved in diverse diseases, with mutation of GPI‐anchor genes mainly causing neurological diseases 16 . The loss of GPI‐Aps are the cause of paroxysmal nocturnal hemoglobinuria 17 …”

Section: Glycosylation and Its Importance In Normal Cellular Functionmentioning

confidence: 99%

The “sugar‐coated bullets” of cancer: Tumor‐derived exosome surface glycosylation from basic knowledge to applications

Lin

Zhou

Tan

2020

Clinical & Translational Med

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Scientific interest in exosomes has exploded in recent decades. In 1990 only three articles were published on exosomes, while over 1,700 have already been published halfway into 2020. 1 While researchers have shown much interest in exosomes since being discovered in 1981, an appreciation of the potential role of glycans in exosome structure and function has emerged only recently. Glycosylation is one of the most common post-translational modification, which functions in many physiological and pathological aspects of cellular function. Many components of exosomes are heavily glycosylated including proteins, lipids, among others. Thus, glycosylation undoubtedly has a great impact on exosome biosynthesis and function. Despite the importance of glycosylation in exosomes and the recent recognition of them as biomarkers for not only malignancies but also other system dysfunction and disease, the characterization of exosome glycans remains understudied. In this review, we discuss glycosylation patterns of exosomes derived from various tissues, their biological features, and potential for various clinical applications. We highlight state-of-the-art knowledge about the fine structure of exosomes, which will allow researchers to reconstruct them by surface modification. These efforts will likely lead to novel disease-related biomarker discovery, purification tagging, and targeted drug transfer for clinical applications in the future.

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Glycan Antigens of Pig Interfering with Xenotransplantation: Three Immune Responses from the Glycans

Kim

2024

Glycoimmunology in Xenotransplantation

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Paroxysmal nocturnal hemoglobinuria without GPI-anchor deficiency

Cited by 6 publications

References 19 publications

Gata1s mutant mice display persistent defects in the erythroid lineage

Gata1s mutant mice display persistent defects in the erythroid lineage

The “sugar‐coated bullets” of cancer: Tumor‐derived exosome surface glycosylation from basic knowledge to applications

Glycan Antigens of Pig Interfering with Xenotransplantation: Three Immune Responses from the Glycans

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