Serum enzymes of collagen synthesis and type III procollagen aminopropeptide in Nigerian patients with sickle cell disease.
Serum immunoreactive prolyl hydroxylase protein (S-IRPH), galactosylhydroxylysyl glucosyltransferase activity (S-GGT), and the aminoterminal propeptide of type III procollagen (S-Pro(III)-N-P) were measured in 20 patients with sickle cell disease and the values were compared with those in 20 apparently healthy Nigerians. The means for the two enzymes and S-Pro(III)-N-P were significantly elevated in the sickle cell disease patients. Significant correlations were found between the values of the two enzymes and the protein (S-Pro(III)-N-P) within the sickle cell disease patients. The data confirm that collagen formation is found in the bone, liver, or other organs of patients with this disease. The measurement of S-GGT and S-Pro(III)-N-P in prospective studies might be helpful in predicting general and hepatic fibrogenesis in sickle cell disease.
D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D000755:Anemia, Sickle Cell / Q000201:enzymology* / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000096:biosynthesis*; D005964:Glucosyltransferases / Q000097:blood*; D006801:Humans; D009549:Nigeria; D010446:Peptide Fragments / Q000097:blood*; D011347:Procollagen / Q000097:blood*; D011393:Procollagen-Proline Dioxygenase / Q000276:immunology*
D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide; D003094:Collagen; D011393:Procollagen-Proline Dioxygenase; D005964:Glucosyltransferases; C023247:UDP glucose-collagen glucosyltransferase; D000410:Alanine Transaminase