Id |
Subject |
Object |
Predicate |
Lexical cue |
T1 |
0-33 |
Sentence |
denotes |
Ehlers Danlos syndrome type VIIB. |
T2 |
34-234 |
Sentence |
denotes |
Incomplete cleavage of abnormal type I procollagen by N-proteinase in vitro results in the formation of copolymers of collagen and partially cleaved pNcollagen that are near circular in cross-section. |
T3 |
235-394 |
Sentence |
denotes |
We have shown that a child with Ehlers Danlos syndrome (EDS) type VII has a G to A transition at the first nucleotide of intron 6 in one of her COL1A2 alleles. |
T4 |
395-481 |
Sentence |
denotes |
Half of the cDNA clones prepared from the proband's pro alpha 2(I) mRNA lacked exon 6. |
T5 |
482-693 |
Sentence |
denotes |
The type I procollagen secreted by the proband's dermal fibroblasts in culture was purified, and collagen fibrils were generated in vitro by cleavage of the procollagen with the procollagen N- and C-proteinases. |
T6 |
694-808 |
Sentence |
denotes |
Incubation of the procollagen with N-proteinase resulted in a 1:1 mixture of pCcollagen and uncleaved procollagen. |
T7 |
809-954 |
Sentence |
denotes |
Incubation of this mixture with C-proteinase generated collagen and abnormal pNcollagen (pNcollagen-ex6) that readily copolymerized into fibrils. |
T8 |
955-1222 |
Sentence |
denotes |
By electron microscopy these fibrils resembled the hieroglyphic fibrils seen in the N-proteinase-deficient skin of dermatosparactic animals and humans and were distinct from the near circular cross-section fibrils seen in the tissues of individuals with EDS type VII. |
T9 |
1223-1398 |
Sentence |
denotes |
Further incubation of the hieroglyphic fibrils with N-proteinase resulted in partial cleavage of the pNcollagen-ex6 in which the abnormal pN alpha 2(I) chains remained intact. |
T10 |
1399-1475 |
Sentence |
denotes |
These fibrils were not hieroglyphic but were near circular in cross-section. |
T11 |
1476-1661 |
Sentence |
denotes |
Fibrils formed from collagen and pNcollagen-ex6 that had been partially cleaved with elevated amounts of N-proteinase prior to fibril formation were also near circular in cross-section. |
T12 |
1662-1832 |
Sentence |
denotes |
The results are consistent with a model of collagen fibril formation in which the intact N-propeptides are located exclusively at the surface of the hieroglyphic fibrils. |
T13 |
1833-1963 |
Sentence |
denotes |
Partial cleavage of the pNcollagen-ex6 by N-proteinase allows the N-propeptides to be incorporated within the body of the fibrils. |
T14 |
1964-2108 |
Sentence |
denotes |
The model provides an explanation for the morphology and molecular composition of collagen fibrils in the tissues of patients with EDS type VII. |
T1 |
0-33 |
Sentence |
denotes |
Ehlers Danlos syndrome type VIIB. |
T2 |
34-234 |
Sentence |
denotes |
Incomplete cleavage of abnormal type I procollagen by N-proteinase in vitro results in the formation of copolymers of collagen and partially cleaved pNcollagen that are near circular in cross-section. |
T3 |
235-394 |
Sentence |
denotes |
We have shown that a child with Ehlers Danlos syndrome (EDS) type VII has a G to A transition at the first nucleotide of intron 6 in one of her COL1A2 alleles. |
T4 |
395-481 |
Sentence |
denotes |
Half of the cDNA clones prepared from the proband's pro alpha 2(I) mRNA lacked exon 6. |
T5 |
482-693 |
Sentence |
denotes |
The type I procollagen secreted by the proband's dermal fibroblasts in culture was purified, and collagen fibrils were generated in vitro by cleavage of the procollagen with the procollagen N- and C-proteinases. |
T6 |
694-808 |
Sentence |
denotes |
Incubation of the procollagen with N-proteinase resulted in a 1:1 mixture of pCcollagen and uncleaved procollagen. |
T7 |
809-954 |
Sentence |
denotes |
Incubation of this mixture with C-proteinase generated collagen and abnormal pNcollagen (pNcollagen-ex6) that readily copolymerized into fibrils. |
T8 |
955-1222 |
Sentence |
denotes |
By electron microscopy these fibrils resembled the hieroglyphic fibrils seen in the N-proteinase-deficient skin of dermatosparactic animals and humans and were distinct from the near circular cross-section fibrils seen in the tissues of individuals with EDS type VII. |
T9 |
1223-1398 |
Sentence |
denotes |
Further incubation of the hieroglyphic fibrils with N-proteinase resulted in partial cleavage of the pNcollagen-ex6 in which the abnormal pN alpha 2(I) chains remained intact. |
T10 |
1399-1475 |
Sentence |
denotes |
These fibrils were not hieroglyphic but were near circular in cross-section. |
T11 |
1476-1661 |
Sentence |
denotes |
Fibrils formed from collagen and pNcollagen-ex6 that had been partially cleaved with elevated amounts of N-proteinase prior to fibril formation were also near circular in cross-section. |
T12 |
1662-1832 |
Sentence |
denotes |
The results are consistent with a model of collagen fibril formation in which the intact N-propeptides are located exclusively at the surface of the hieroglyphic fibrils. |
T13 |
1833-1963 |
Sentence |
denotes |
Partial cleavage of the pNcollagen-ex6 by N-proteinase allows the N-propeptides to be incorporated within the body of the fibrils. |
T14 |
1964-2108 |
Sentence |
denotes |
The model provides an explanation for the morphology and molecular composition of collagen fibrils in the tissues of patients with EDS type VII. |