thymosin and Syndrome

Congenitally athymic nude mice (nu/nu) are presented as a model for the study of cell-mediated immunologic deficiencies. These mice possess a vestigial thymus which is incapable of producing mature T-cells as shown by a decreased lymphocyte population carrying the theta antigen and depleted 'thymus-dependent' areas in their peripheral lymphoid organs. However, they have T-cell precursors in their bone marrow. Nude mice lack 'thymosin', a thymic factor secreted by the epithelial cells of the thymus. They also have impaired T-cell functions as demonstrated by an absence of delayed hypersensitivity reactions, an inability to reject skin allografts and a decreased antibody response to some T-dependent antigens. But their response to T-independent antigens is normal. The presence of circulating auto-antibodies and immune-complex type glomerulonephritis in nude mice reinforces the hypothesis that spontaneous autoimmunity and B-cell hyperactivity are associated with T-cell deficiency. On the other hand, they are not especially susceptible to spontaneous tumor development. Correlations are made between the pathologic findings in nude mice and some congenital cellular immunologic disorders in man.

Topics: Agammaglobulinemia; Animals; Antigens; Ataxia Telangiectasia; Autoantibodies; Disease Models, Animal; Graft Rejection; Immunity, Cellular; Immunoglobulin G; Immunoglobulin M; Immunologic Deficiency Syndromes; Mice; Mice, Nude; Neoplasm Transplantation; Neoplasms, Experimental; Spleen; Syndrome; T-Lymphocytes; Thymosin; Thymus Gland; Wiskott-Aldrich Syndrome

Five fragments from hexapeptide to decapeptide, corresponding to positions 16-25 of thymosin beta 9, were synthesized and their effects on low E-rosette-forming capacity with sheep erythrocytes of cells from lupus nephritis patients were compared with that of the undecapeptide (positions 16-26) of thymosin beta 9 by taking synthetic thymosin beta 9 as a standard. Two of the fragments (16-25 and 18-25) exhibited higher activity than that of the parent peptide (16-26). The other three sequences (17-25, 19-25, and 20-25) had no effect at concentrations as high as 10(-4) M.

Topics: Amino Acid Sequence; Glomerulonephritis; Humans; Immune Complex Diseases; Indicators and Reagents; Lupus Erythematosus, Systemic; Peptide Fragments; Rosette Formation; Syndrome; Thymosin

A child with the cerebro-hepato-renal syndrome of Zellweger, who was originally diagnosed as having the DiGeorge syndrome, was studied and transplanted unsuccessfully with cultured thymus. The pertinent literature is reviewed and the importance of distinguishing the two disorders emphasized. Autopsy studies reveal that transplanted cultured thymic fragments can attract lymphoid aggregates as early as 2 wk after transplantation.

Topics: Abnormalities, Multiple; Brain; Diagnosis, Differential; DiGeorge Syndrome; Humans; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Kidney; Liver; Lymphoid Tissue; Male; Mitochondria; Rosette Formation; Syndrome; Thymosin; Thymus Gland

Topics: Adolescent; Agammaglobulinemia; Ataxia Telangiectasia; Burkitt Lymphoma; Candidiasis, Cutaneous; Child; Child, Preschool; Chronic Disease; Female; Humans; Immunologic Deficiency Syndromes; Lymphatic Diseases; Male; Middle Aged; Syndrome; T-Lymphocytes; Thymosin; Thymus Gland; Thymus Hormones

Topics: Adult; Animals; Antilymphocyte Serum; Bone Marrow; Bone Marrow Cells; Cattle; Cell Differentiation; Cell Separation; Cells, Cultured; Concanavalin A; Epitopes; Erythrocytes; Humans; Immune Adherence Reaction; Immunologic Deficiency Syndromes; Infant; Lectins; Male; Mitomycins; Parathyroid Glands; Sheep; Spleen; Syndrome; T-Lymphocytes; Thymidine; Thymosin; Thymus Extracts; Thymus Gland; Tissue Extracts; Transfer Factor; Tritium

Topics: Adult; Agammaglobulinemia; Ataxia Telangiectasia; Child; Dose-Response Relationship, Drug; Female; Histocompatibility Antigens; Humans; Immune Adherence Reaction; Immunoglobulin A; Immunoglobulin G; Immunologic Deficiency Syndromes; Infant; Liver; Mosaicism; Nasopharyngeal Neoplasms; Parathyroid Glands; Respiratory Tract Infections; Spleen; Syndrome; T-Lymphocytes; Thymosin; Thymus Extracts; Thymus Gland; Tissue Extracts; Transplantation, Homologous; Wiskott-Aldrich Syndrome