Biomaterial Database

Severe combined immunodeficiency disease: a model of T-cell dysfunction. 1978

H M Dosch, and J W Lee, and E W Gelfand, and J A Falk

Studies in three patients with severe combined immunodeficiency disease (SCID) and normal adenosine deaminase demonstrated that the combined defect of both T- and B-lymphocyte function may reflect the lack of normal maturation of thymic epithelial cells. This results in the failure of initiation of T-cell differentiation and consequent failure of T-cell dependent maturation of B-lymphocytes to an antibody-secreting stage. SCID B lymphocytes were shown to be capable of generating a specific IgM-antibody response to two T-cell-dependent antigens in vitro under either of the following conditions: (a) provision of autologous T-helper cells which were induced following incubation of precursor cells on monolayers of cultured human thymic epithelium or (b) in the presence of allogeneic T-helper cells. Specific IgM anti-ovalbumin (OA) responses were also generated in the absence of provided T-helper cells when the antigen was insolubilized (Sepharose-OA). The antibody-secreting cells and their circulating precursors carried surface IgM, HLA and Ia-like determinants and proliferated in response to antigen. Identification of this form of SCID may be important when considering therapy and provides an excellent model for the study of the T-cell-dependent acquisition and expression of B-cell immunity.

UI	MeSH Term	Description	Entries
D007153	Immunologic Deficiency Syndromes	Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.	Antibody Deficiency Syndrome,Deficiency Syndrome, Immunologic,Deficiency Syndromes, Antibody,Deficiency Syndromes, Immunologic,Immunologic Deficiency Syndrome,Immunological Deficiency Syndromes,Antibody Deficiency Syndromes,Deficiency Syndrome, Antibody,Deficiency Syndrome, Immunological,Deficiency Syndromes, Immunological,Immunological Deficiency Syndrome,Syndrome, Antibody Deficiency,Syndrome, Immunologic Deficiency,Syndrome, Immunological Deficiency,Syndromes, Antibody Deficiency,Syndromes, Immunologic Deficiency,Syndromes, Immunological Deficiency
D008211	Lymphocyte Cooperation	T-cell enhancement of the B-cell response to thymic-dependent antigens.	Cooperation, Lymphocyte,Cooperations, Lymphocyte,Lymphocyte Cooperations
D008954	Models, Biological	Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.	Biological Model,Biological Models,Model, Biological,Models, Biologic,Biologic Model,Biologic Models,Model, Biologic
D002478	Cells, Cultured	Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.	Cultured Cells,Cell, Cultured,Cultured Cell
D004847	Epithelial Cells	Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.	Adenomatous Epithelial Cells,Columnar Glandular Epithelial Cells,Cuboidal Glandular Epithelial Cells,Glandular Epithelial Cells,Squamous Cells,Squamous Epithelial Cells,Transitional Epithelial Cells,Adenomatous Epithelial Cell,Cell, Adenomatous Epithelial,Cell, Epithelial,Cell, Glandular Epithelial,Cell, Squamous,Cell, Squamous Epithelial,Cell, Transitional Epithelial,Cells, Adenomatous Epithelial,Cells, Epithelial,Cells, Glandular Epithelial,Cells, Squamous,Cells, Squamous Epithelial,Cells, Transitional Epithelial,Epithelial Cell,Epithelial Cell, Adenomatous,Epithelial Cell, Glandular,Epithelial Cell, Squamous,Epithelial Cell, Transitional,Epithelial Cells, Adenomatous,Epithelial Cells, Glandular,Epithelial Cells, Squamous,Epithelial Cells, Transitional,Glandular Epithelial Cell,Squamous Cell,Squamous Epithelial Cell,Transitional Epithelial Cell
D006462	Hemolytic Plaque Technique	A method to identify and enumerate cells that are synthesizing ANTIBODIES against ANTIGENS or HAPTENS conjugated to sheep RED BLOOD CELLS. The sheep red blood cells surrounding cells secreting antibody are lysed by added COMPLEMENT producing a clear zone of HEMOLYSIS. (From Illustrated Dictionary of Immunology, 3rd ed)	Jerne's Plaque Technique,Hemolytic Plaque Technic,Jerne's Plaque Technic,Hemolytic Plaque Technics,Hemolytic Plaque Techniques,Jerne Plaque Technic,Jerne Plaque Technique,Jernes Plaque Technic,Jernes Plaque Technique,Plaque Technic, Hemolytic,Plaque Technic, Jerne's,Plaque Technics, Hemolytic,Plaque Technique, Hemolytic,Plaque Technique, Jerne's,Plaque Techniques, Hemolytic,Technic, Hemolytic Plaque,Technic, Jerne's Plaque,Technics, Hemolytic Plaque,Technique, Hemolytic Plaque,Technique, Jerne's Plaque,Techniques, Hemolytic Plaque
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000917	Antibody Formation	The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.	Antibody Production,Antibody Response,Antibody Responses,Formation, Antibody,Production, Antibody,Response, Antibody,Responses, Antibody
D001402	B-Lymphocytes	Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.	B-Cells, Lymphocyte,B-Lymphocyte,Bursa-Dependent Lymphocytes,B Cells, Lymphocyte,B Lymphocyte,B Lymphocytes,B-Cell, Lymphocyte,Bursa Dependent Lymphocytes,Bursa-Dependent Lymphocyte,Lymphocyte B-Cell,Lymphocyte B-Cells,Lymphocyte, Bursa-Dependent,Lymphocytes, Bursa-Dependent
D012397	Rosette Formation	The in vitro formation of clusters consisting of a cell (usually a lymphocyte) surrounded by antigenic cells or antigen-bearing particles (usually erythrocytes, which may or may not be coated with antibody or antibody and complement). The rosette-forming cell may be an antibody-forming cell, a memory cell, a T-cell, a cell bearing surface cytophilic antibodies, or a monocyte possessing Fc receptors. Rosette formation can be used to identify specific populations of these cells.	Immunocytoadherence,Formation, Rosette,Formations, Rosette,Immunocytoadherences,Rosette Formations