Dermatosparaxis
Mostrando 1-6 de 6 artigos, teses e dissertações.
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1. Dermatosparaxis em dois cordeiros White Dorper no Brasil: relato de caso
A dermatosparaxis é uma doença genética que afeta a maturação do colágeno. Essa doença é caracterizada por redução acentuada da resistência das fibras colágenas que leva à fragilidade da pele. Esse é o primeiro relato de dermatosparaxis em ovinos no Brasil. Clinicamente, dois cordeiros da raça White Dorper tiveram múltiplas lacerações na pe
Arq. Bras. Med. Vet. Zootec.. Publicado em: 2015-06
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2. Cell-Free Synthesis of Procollagen: L-929 Fibroblasts as a Cellular Model for Dermatosparaxis
A cell-free system that actively synthesizes collagen was prepared from L-929 fibroblasts. Chromatographic and electrophoretic techniques were used to demonstrate that the only collagenous products are pro α1 and pro α2 chains. The collagen synthesized by the cell-free system was also compared to the collagen extracted from the cells. The cellular collagen
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3. Procollagen Peptidase: An Enzyme Excising the Coordination Peptides of Procollagen
A heritable connective tissue disorder of cattle, dermatosparaxis, is characterized by an extreme fragility of the skin and the presence of additional peptides at the N-terminal extremities of the collagen α chains, p-α1 and p-α2.
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4. Low rate of procollagen conversion in dermatosparactic sheep fibroblasts is paralleled by increased synthesis of type I and type III collagens.
Dermatosparaxis is a genetic defect found in humans and animals, which affects the conversion of procollagen to collagen and aminopropeptides . Under cell culture conditions a reduction of the release of aminopropeptides is paralleled by an increased synthesis of collagens type I and III. This fact provides further evidence for a regulatory involvement of am
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5. Procollagen alpha 2(I) mRNA in dermatosparactic fibroblasts: evidence for post-transcriptional regulation.
Dermatosparaxis is a genetic defect of connective tissues found in man and animals. Dermatosparactic sheep fibroblasts fail to cleave off the aminopropeptide of procollagen, this makes the feedback regulation less effective and leads to an elevated production of collagen. We have isolated the total RNA from dermatosparactic and normal sheep fibroblasts and t
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6. cDNA cloning and expression of bovine procollagen I N-proteinase: A new member of the superfamily of zinc-metalloproteinases with binding sites for cells and other matrix components
Procollagen N-proteinase (EC 3.4.24.14) cleaves the amino-propeptides in the processing of type I and type II procollagens to collagens. Deficiencies of the enzyme cause dermatosparaxis in cattle and sheep, and they cause type VIIC Ehlers–Danlos syndrome in humans, heritable disorders characterized by accumulation of pNcollagen and severe skin fragility. A
The National Academy of Sciences of the USA.