HYDROXYLYSINE DEFICIENT COLLAGEN IN A FLOPPY BABY
نویسندگان
چکیده
منابع مشابه
Abnormal Properties of Collagen Lysyl Hydroxylase from Skin Fibroblasts of Siblings with Hydroxylysine - Deficient Collagen Rum
Received for publication 30 June 1975 and in revised form 25 August 1975. part due to differences in cofactor concentrations and in rate and sequence of events in collagen synthesis in different tissues.
متن کاملAbnormal properties of collagen lysyl hydroxylase from skin fibroblasts of siblings with hydroxylysine-deficient collagen.
Skin fibroblasts from two siblings with hydroxylysine-deficient collagen collagen (Ehlers-Danlos syndrome, type VI) contained normal levels of collagen prolyl hydroxylase activity but were markedly deficient in collagen lysyl hydroxylase activity. The deficiency was evident in all fractions of cell lysates, in low and high ionic strength buffers, and in detergent. Assays of mixtures of wild-typ...
متن کاملThe formation of collagen hydroxylysine.
The sole source of hydroxylysine in the skin collagen of rats has been found to be lysine (1, 2). Rats fed W-labeled lysine showed the same Cl4 specific activity in the hydroxylysine as in the lysine isolated from skin collagen (1). Labeled hydroxylysine, when injected into rats, was not incorporated into the collagen (2). To find whether hydroxylation of lysine is completed at the time of inco...
متن کاملLysyl-protocollagen hydroxylase deficiency in fibroblasts from siblings with hydroxylysine-deficient collagen.
Cell culture studies were performed on members of a family in which two sisters, ages 9 and 12, have a similar disorder characterized clinically by severe scoliosis, joint laxity and recurrent dislocations, hyperextensible skin, and thin scars. The skin collagen from the sisters was markedly deficient in hydroxylysine, but other amino acids were present in normal amounts. Hydroxylysine in colla...
متن کاملThe source and state of the hydroxylysine of collagen. II. Failure of free hydroxylysine to serve as a source of the hydroxylysine or lysine of collagen.
The amino acid 5-hydroxylysine, first isolated from gelatin (2), has been found absent in all types of proteins other than collagen that have been examined (3). It is not an essential amino acid for animal nutrition (4), and hence must be formed from other material in the animal body. Previous papers from this laboratory (5, 6) have shown that lysine is the chief source of the hydroxylysine inc...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1977
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-197704000-01154