X-linked Severe combined immunodeficiency

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Safety Modality for X-linked Severe Combined Immunodeficiency Gene Therapy

Abbreviations: SCID-X1: X-linked Severe Combined Immunodeficiency; γc: Cytokine Receptor Common Gamma Chain; HSC: Hematopoietic Stem Cells; ab: Antibody; wt: Wild-type; NK cell: Natural Killer Cell; HLA: Human Leukocyte Antigen; SIN: Selfinactivating; HSV-TK: Thymidine kinase of Herpes Simplex Virus; ER: Endoplasmatic Reticulum; MFI: Mean Fluorescence Intensity; CDC: Complement-dependent Cytoto...

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Efficacy of gene therapy for X-linked severe combined immunodeficiency.

BACKGROUND The outcomes of gene therapy to correct congenital immunodeficiencies are unknown. We reviewed long-term outcomes after gene therapy in nine patients with X-linked severe combined immunodeficiency (SCID-X1), which is characterized by the absence of the cytokine receptor common gamma chain. METHODS The nine patients, who lacked an HLA-identical donor, underwent ex vivo retrovirus-me...

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Bone marrow transplantation for canine X-linked severe combined immunodeficiency.

Canine X-linked severe combined immunodeficiency (XSCID) is due to mutations in the common gamma chain which is a subunit of the receptors of IL-2, IL-4, IL-7, IL-9 and IL-15. Bone marrow transplantation (BMT) of human XSCID patients without pretransplant conditioning (cytoablation) results in engraftment of donor T-cells and reconstitution of T-cell function but engraftment of few, if any, don...

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Retroviral-mediated gene correction for X-linked severe combined immunodeficiency.

X-linked severe combined immunodeficiency (XSCID) is a lethal disease caused by a defect in the gene encoding the common gamma chain (gamma-c) of the receptor for interleukin-2 (IL-2), IL-4, IL-7, IL-9, and IL-15. Allogeneic bone marrow transplantation, the current therapy of choice for this defect, is often complicated by graft-versus-host disease and/or incomplete reconstitution of B-lymphocy...

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Generation of Knockout Rats with X-Linked Severe Combined Immunodeficiency (X-SCID) Using Zinc-Finger Nucleases

BACKGROUND Although the rat is extensively used as a laboratory model, the inability to utilize germ line-competent rat embryonic stem (ES) cells has been a major drawback for studies that aim to elucidate gene functions. Recently, zinc-finger nucleases (ZFNs) were successfully used to create genome-specific double-stranded breaks and thereby induce targeted gene mutations in a wide variety of ...

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ژورنال

عنوان ژورنال: Japanese Journal of Clinical Immunology

سال: 2002

ISSN: 0911-4300,1349-7413

DOI: 10.2177/jsci.25.140