Serum enzymes of collagen synthesis and type III procollagen amino-propeptide in Nigerian patients with sickle cell disease.
Serum immunoreactive prolyl hydroxylase protein, galactosylhydroxylysyl glucosyltransferase activity and the aminoterminal propeptide of type III procollagen 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 serum aminoterminal propeptide of type III procollagen were significantly higher in the sickle cell disease patients. Significant correlations were found between the values for the two enzymes and the protein serum aminoterminal propeptide of type III procollagen within the sickle cell disease patients. The data confirm that collagen formation is found in the liver, bone and other organs of patients with this disease. The measurement of serum immunoreactive prolyl hydroxylase protein, serum galactosylhydroxylysyl glucosyltransferase activity and serum aminoterminal propeptide of type III procollagen in prospective studies might be helpful in predicting hepatic, bone or diffuse fibrogenesis in sickle cell disease.
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000096:biosynthesis*; D005260:Female; D005964:Glucosyltransferases / Q000097:blood; D006801:Humans; D008297:Male; D011347:Procollagen / Q000378:metabolism*; D011393:Procollagen-Proline Dioxygenase / Q000097:blood; D011863:Radioimmunoassay
D011347:Procollagen; D003094:Collagen; D011393:Procollagen-Proline Dioxygenase; D005964:Glucosyltransferases; C023247:UDP glucose-collagen glucosyltransferase