Lymphocyte ecto-5'-nucleotidase deficiency in agammaglobulinemia.
Fresh peripheral blood lymphocytes from eight patients with congenital agammaglobulinemia demonstrate reduced ecto-5'-nucleotidase activity when compared to the mean activity of normal subjects and patients with other forms of immunoglobulin deficiency. A specific defect of ecto-5'-nucleotidase is further suggested by normal values for lymphocyte ecto-adenosinetriphosphatase and ecto-nonspecific phosphatase. The data provide evidence for an enzyme deficiency in this X-linked, B lymphocyte deficiency syndrome.
D000361:Agammaglobulinemia / Q000201:enzymology* / Q000235:genetics; D002462:Cell Membrane / Q000201:enzymology; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D006863:Hydrogen-Ion Concentration; D017098:IgA Deficiency; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D009708:Nucleotidases / Q000097:blood / Q000172:deficiency*; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000276:immunology; D014960:X Chromosome
D009708:Nucleotidases