Soluble sphingomyelinase from human urine as antigen for obtaining anti-sphingomyelinase antibodies.
A soluble form of lysosomal sphingomyelinase was partially purified from human urine using concanavalin A-Sepharose 4B, Sephadex G-100 and octyl-Sepharose 4B chromatography. The octyl-Sepharose 4B eluate was used to immunise a rabbit. The antiserum obtained was able to precipitate about 70% of the sphingomyelinase activity present in urine from control subjects. Both the immunoprecipitable and non-precipitable activities were found to be deficient in urine from patients with Niemann-Pick disease Type A and Type B. In contrast, both activities were present in urine from patients with Niemann-Pick disease Type C. The antiserum was able to precipitate about 80% of the sphingomyelinase activity present in an aqueous extract of placenta.
Weitz
G
G
Driessen
M
M
Brouwer-Kelder
E M
EM
Sandhoff
K
K
Barranger
J A
JA
Tager
J M
JM
Schram
A W
AW
D000818:Animals; D000906:Antibodies / Q000276:immunology*; D000941:Antigens / Q000276:immunology; D002845:Chromatography; D005260:Female; D005347:Fibroblasts / Q000276:immunology; D006801:Humans; D007163:Immunosorbent Techniques; D009542:Niemann-Pick Diseases / Q000201:enzymology; D010727:Phosphoric Diester Hydrolases / Q000652:urine*; D010920:Placenta / Q000201:enzymology; D011247:Pregnancy; D011817:Rabbits / Q000276:immunology; D013108:Sphingomyelin Phosphodiesterase / Q000276:immunology / Q000652:urine*; D013154:Spleen / Q000201:enzymology
D000906:Antibodies; D000941:Antigens; D010727:Phosphoric Diester Hydrolases; D013108:Sphingomyelin Phosphodiesterase