A mild form of ovine dermatosparaxis.
Dermatosparaxis is an inherited disease which results from a deficiency in procollagen aminopropeptidase activity. A form of this disease in sheep has been examined which is much milder than the previously reported instance in sheep. Partially processed pN alpha 1 and pN alpha 2 chains are present in the skin of affected animals and have been characterized by gel electrophoresis, amino acid analysis, susceptibility to collagenase digestions and by segment-long-spacing aggregate formations. Fully processed collagen is also present in the skin of affected animals indicating that some aminopropeptidase activity is present. Extracts from the skin of affected animals were shown to contain an aminopropeptidase activity which was about 25% of the activity found in similar extracts from normal animals.
D000596:Amino Acids / Q000032:analysis; D000818:Animals; D002848:Chromatography, DEAE-Cellulose; D003094:Collagen / Q000032:analysis; D003240:Connective Tissue Diseases / Q000201:enzymology / Q000473:pathology / Q000662:veterinary*; D004591:Electrophoresis, Polyacrylamide Gel; D010450:Endopeptidases / Q000172:deficiency*; D011348:Procollagen N-Endopeptidase / Q000172:deficiency*; D012756:Sheep; D012757:Sheep Diseases / Q000201:enzymology* / Q000473:pathology; D012867:Skin / Q000032:analysis; D012871:Skin Diseases / Q000201:enzymology / Q000473:pathology / Q000662:veterinary*
D000596:Amino Acids; D003094:Collagen; D010450:Endopeptidases; D011348:Procollagen N-Endopeptidase