title: Serum alkaline phosphatase in hypophosphatasia.
abstract: It is recognized that serum alkaline phosphatase may reflect enzyme contributions from bone, liver, and intestine. We have investigated serum alkaline phosphatases in two siblings with hypophosphatasia. After administration of long-chain triglycerides, the major alkaline phosphatase component of their sera was shown to be of intestinal origin on the basis of inhibition by l-phenylalanine. Starch block electrophoresis suggested that there were other regions of l-phenylalanine-sensitive alkaline phosphatase in addition to the major slow-moving intestinal band. Medium-chain triglycerides which are absorbed by the portal route did not cause a similar augmentation of intestinal alkaline phosphatase activity. These studies indicate that serum levels of intestinal alkaline phosphatase are increased normally after long-chain fat feeding in hypophosphatasia and may be the major component of total serum alkaline phosphatase activity.
mesh_terms: D000469:Alkaline Phosphatase / Q000097:blood*; D001842:Bone and Bones / Q000201:enzymology; D004041:Dietary Fats / Q000494:pharmacology; D004586:Electrophoresis; D005260:Female; D006358:Hot Temperature; D006801:Humans; D007014:Hypophosphatasia / Q000201:enzymology* / Q000235:genetics; D007223:Infant; D007231:Infant, Newborn; D007422:Intestines / Q000201:enzymology*; D007962:Leukocytes / Q000201:enzymology; D008297:Male; D010649:Phenylalanine; D010714:Phosphatidylethanolamines / Q000652:urine; D011489:Protein Denaturation; D013268:Stimulation, Chemical; D014280:Triglycerides / Q000494:pharmacology
chemical_list: D004041:Dietary Fats; D010714:Phosphatidylethanolamines; D014280:Triglycerides; D010649:Phenylalanine; D000469:Alkaline Phosphatase