|
rdf:type |
|
|
lifeskim:mentions |
|
|
pubmed:issue |
3
|
|
pubmed:dateCreated |
1993-4-13
|
|
pubmed:abstractText |
We studied the radiosensitivity of granulocyte macrophage colony-forming units (GM-CFU) in patients with a severe combined immunodeficiency (SCID). Three patients lacking both mature T and B cells showed a twofold higher GM-CFU radiosensitivity calculated as the DO value (dose required to reduce survival to 37%), and an identical observation was made with fibroblasts from one of these patients. A patient with an SCID with hypereosinophilia, i.e., Omenn's syndrome characterized by extremely restricted T cell heterogeneity and a lack of B cells, also showed abnormal GM-CFU radiosensitivity. In contrast, GM-CFU from a patient lacking only T cells (X-linked form of SCID) showed normal GM-CFU radiosensitivity. These data further support the similarity between human T(-) B(-) SCID and the murine acid mutation characterized by a defect in T cell receptor and immunoglobulin gene rearrangement, and by an abnormal double-strand DNA break repair function. In addition, they strongly suggest that the Omenn's immunodeficiency syndrome may be a leaky T(-)B(-) SCID phenotype as previously indicated by the coexistence of the two phenotypes in siblings.
|
|
pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1196376,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1676369,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1693357,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1709732,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1804782,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1869046,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1940786,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-1971143,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2010548,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2196911,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2215662,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2896355,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-29085,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2908877,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-2991773,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-3280724,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-3670329,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-3873654,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-5412675,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-571047,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-6307430,
http://linkedlifedata.com/resource/pubmed/commentcorrection/8450050-6823332
|
|
pubmed:language |
eng
|
|
pubmed:journal |
|
|
pubmed:citationSubset |
AIM
|
|
pubmed:chemical |
|
|
pubmed:status |
MEDLINE
|
|
pubmed:month |
Mar
|
|
pubmed:issn |
0021-9738
|
|
pubmed:author |
|
|
pubmed:issnType |
Print
|
|
pubmed:volume |
91
|
|
pubmed:owner |
NLM
|
|
pubmed:authorsComplete |
Y
|
|
pubmed:pagination |
1214-8
|
|
pubmed:dateRevised |
2009-11-18
|
|
pubmed:meshHeading |
pubmed-meshheading:8450050-Bone Marrow,
pubmed-meshheading:8450050-Cell Survival,
pubmed-meshheading:8450050-Cells, Cultured,
pubmed-meshheading:8450050-Colony-Forming Units Assay,
pubmed-meshheading:8450050-DNA,
pubmed-meshheading:8450050-Dose-Response Relationship, Radiation,
pubmed-meshheading:8450050-Fibroblasts,
pubmed-meshheading:8450050-Genes, Recessive,
pubmed-meshheading:8450050-Granulocytes,
pubmed-meshheading:8450050-Hematopoietic Stem Cells,
pubmed-meshheading:8450050-Humans,
pubmed-meshheading:8450050-Immunologic Deficiency Syndromes,
pubmed-meshheading:8450050-Infant,
pubmed-meshheading:8450050-Macrophages,
pubmed-meshheading:8450050-Male,
pubmed-meshheading:8450050-Reference Values,
pubmed-meshheading:8450050-Restriction Mapping,
pubmed-meshheading:8450050-Skin,
pubmed-meshheading:8450050-T-Lymphocytes
|
|
pubmed:year |
1993
|
|
pubmed:articleTitle |
Increased radiosensitivity of granulocyte macrophage colony-forming units and skin fibroblasts in human autosomal recessive severe combined immunodeficiency.
|
|
pubmed:affiliation |
Institut National de la Santé et de la Recherche Médicale U 132, Hôpital Necker-Enfants Malades, Paris, France.
|
|
pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|
