X-Linked Combined Immunodeficiency Diseases (X Linked Severe Combined Immunodeficiency)

Forms of combined immunodeficiency caused by mutations in the gene for INTERLEUKIN RECEPTOR COMMON GAMMA SUBUNIT. Both severe and non-severe subtypes of the disease have been identified.
Also Known As:
X Linked Severe Combined Immunodeficiency; Immunodeficiency 4; Immunodeficiency Diseases, X-Linked Combined; X-Linked Combined Immunodeficiency; X-Linked Immunodeficiency Disease; X-Linked Immunodeficiency Syndrome; Combined Immunodeficiencies, X-Linked; Combined Immunodeficiency, X-Linked; Immunodeficiency Disease, X-Linked; Immunodeficiency Diseases, X Linked Combined; Immunodeficiency Diseases, X-Linked; Immunodeficiency Syndrome, X-Linked; Immunodeficiency Syndromes, X-Linked; Immunodeficiency, X-Linked Combined; Syndromes, X-Linked Immunodeficiency; X Linked Combined Immunodeficiency; X Linked Combined Immunodeficiency Diseases; X Linked Immunodeficiency Disease; X Linked Immunodeficiency Syndrome; X-Linked Combined Immunodeficiencies; X-Linked Immunodeficiency Diseases; X-Linked Immunodeficiency Syndromes; X-Linked Severe Combined Immunodeficiency
Networked: 198 relevant articles (7 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Chronic Granulomatous Disease
2. Wiskott-Aldrich Syndrome (Syndrome, Wiskott-Aldrich)
3. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
4. Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome)
5. T-Cell Leukemia (Leukemia, T Cell)


1. Thrasher, Adrian J: 9 articles (10/2014 - 04/2002)
2. Felsburg, Peter J: 8 articles (03/2015 - 01/2002)
3. Malech, Harry L: 8 articles (03/2015 - 07/2002)
4. Leonard, Warren J: 8 articles (10/2011 - 11/2002)
5. Hartnett, Brian J: 7 articles (07/2011 - 01/2002)
6. Moore, Peter F: 6 articles (01/2014 - 04/2006)
7. Henthorn, Paula S: 6 articles (07/2011 - 01/2002)
8. De Ravin, Suk See: 5 articles (03/2015 - 07/2007)
9. Kumaki, Satoru: 5 articles (09/2013 - 09/2002)
10. Sorrentino, Brian P: 4 articles (03/2015 - 05/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to X-Linked Combined Immunodeficiency Diseases:
1. Severe combined immunodeficiency due to adenosine deaminase deficiencyIBA
2. Adenosine DeaminaseIBA
3. Interleukin-2 (IL2)IBA
4. adenine deaminaseIBA
5. X-linked Severe combined immunodeficiencyIBA
6. Complementary DNA (cDNA)IBA
06/12/2014 - "We demonstrate the therapeutic potential of our strategy by targeting a corrective complementary DNA into the IL2RG gene of HSCs from healthy donors and a subject with X-linked severe combined immunodeficiency (SCID-X1). "
12/01/2004 - "Eight of 10 infants with X-linked severe combined immunodeficiency who received autologous hematopoietic stem cells transduced with a retroviral vector carrying the IL2RG complementary DNA achieved immune reconstitution. "
02/01/2004 - "In this study, an MND-IL-2R vector containing IL-2Rc gamma cDNA to treat X-linked severe combined immunodeficiency (X-SCID) was constructed from an MND vector that was modified by substituting the myeloproliferative sarcoma virus (MPSV) enhancer for that of MoMLV, deleting the negative control region located in the long terminal repeat (LTR) as an enhancer, and replacing the primer binding site (PBS) of MoMLV with the PBS of the endogenous murine retrovirus dl587rev. "
10/09/2014 - "In previous clinical trials involving children with X-linked severe combined immunodeficiency (SCID-X1), a Moloney murine leukemia virus-based γ-retrovirus vector expressing interleukin-2 receptor γ-chain (γc) complementary DNA successfully restored immunity in most patients but resulted in vector-induced leukemia through enhancer-mediated mutagenesis in 25% of patients. "
06/01/1994 - "The complete nucleotide sequence of the cDNA indicates that it is related most closely to EMT, a tyrosine kinase of T cells and to the B-cell tyrosine kinase Btk, which is mutated in X-linked agammaglobulinemia (XLA) in humans and X-linked immunodeficiency disease (XID) in mouse. "
7. Interleukin-4 (Interleukin 4)IBA
8. Interleukin-15 (Interleukin 15)IBA
9. Interleukin-13IBA
10. Prostaglandins D (PGD)IBA

Therapies and Procedures

1. Heterologous Transplantation (Xenotransplantation)
2. Stem Cell Transplantation
3. Cord Blood Stem Cell Transplantation
4. Transplants (Transplant)
5. Transplantation (Transplant Recipients)