| Id |
Subject |
Object |
Predicate |
Lexical cue |
| TextSentencer_T1 |
0-229 |
Sentence |
denotes |
Molecular basis of very long chain acyl-CoA dehydrogenase deficiency in three Israeli patients: identification of a complex mutant allele with P65L and K247Q mutations, the former being an exonic mutation causing exon 3 skipping. |
| TextSentencer_T2 |
230-362 |
Sentence |
denotes |
Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a life-threatening disorder of mitochondrial fatty acid beta-oxidation. |
| TextSentencer_T3 |
363-426 |
Sentence |
denotes |
We identified four novel mutations in three unrelated patients. |
| TextSentencer_T4 |
427-526 |
Sentence |
denotes |
All patients had the severe childhood form of VLCAD deficiency with early onset and high mortality. |
| TextSentencer_T5 |
527-719 |
Sentence |
denotes |
Immunoblot analysis revealed that VLCAD protein was undetectable in patients 2 and 3, whereas normal-size VLCAD protein and an aberrant form of VLCAD (4kDa smaller) were detected in patient 1. |
| TextSentencer_T6 |
720-916 |
Sentence |
denotes |
As expected, null mutations were found in patients 2 and 3: patient 2 is homozygous for a frameshift mutation, del 4 bp at 798-801, and patient 3 is homozygous for a nonsense mutation 65C>A(S22X). |
| TextSentencer_T7 |
917-1143 |
Sentence |
denotes |
Patient 1 was homozygous for a complex mutant allele containing two alterations, including a 194C>T transition (P65L) and 739A>C transversion (K247Q); in the case of P65L, the amino acid change does not reduce enzyme activity. |
| TextSentencer_T8 |
1144-1278 |
Sentence |
denotes |
However, the nucleotide change resulted in exon 3 skipping, whereas the latter K247Q mutation had a drastic effect on enzyme activity. |
| TextSentencer_T9 |
1279-1386 |
Sentence |
denotes |
We verified these events by in vivo splicing experiments and transient expression analysis of mutant cDNAs. |
| TextSentencer_T10 |
1387-1466 |
Sentence |
denotes |
The P65L mutation locates 11 bases upstream of a splice donor site of intron 3. |
| TextSentencer_T11 |
1467-1536 |
Sentence |
denotes |
This is an example of an exonic mutation which affects exon-splicing. |
| T1 |
0-229 |
Sentence |
denotes |
Molecular basis of very long chain acyl-CoA dehydrogenase deficiency in three Israeli patients: identification of a complex mutant allele with P65L and K247Q mutations, the former being an exonic mutation causing exon 3 skipping. |
| T2 |
230-362 |
Sentence |
denotes |
Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a life-threatening disorder of mitochondrial fatty acid beta-oxidation. |
| T3 |
363-426 |
Sentence |
denotes |
We identified four novel mutations in three unrelated patients. |
| T4 |
427-526 |
Sentence |
denotes |
All patients had the severe childhood form of VLCAD deficiency with early onset and high mortality. |
| T5 |
527-719 |
Sentence |
denotes |
Immunoblot analysis revealed that VLCAD protein was undetectable in patients 2 and 3, whereas normal-size VLCAD protein and an aberrant form of VLCAD (4kDa smaller) were detected in patient 1. |
| T6 |
720-916 |
Sentence |
denotes |
As expected, null mutations were found in patients 2 and 3: patient 2 is homozygous for a frameshift mutation, del 4 bp at 798-801, and patient 3 is homozygous for a nonsense mutation 65C>A(S22X). |
| T7 |
917-1143 |
Sentence |
denotes |
Patient 1 was homozygous for a complex mutant allele containing two alterations, including a 194C>T transition (P65L) and 739A>C transversion (K247Q); in the case of P65L, the amino acid change does not reduce enzyme activity. |
| T8 |
1144-1278 |
Sentence |
denotes |
However, the nucleotide change resulted in exon 3 skipping, whereas the latter K247Q mutation had a drastic effect on enzyme activity. |
| T9 |
1279-1386 |
Sentence |
denotes |
We verified these events by in vivo splicing experiments and transient expression analysis of mutant cDNAs. |
| T10 |
1387-1466 |
Sentence |
denotes |
The P65L mutation locates 11 bases upstream of a splice donor site of intron 3. |
| T11 |
1467-1536 |
Sentence |
denotes |
This is an example of an exonic mutation which affects exon-splicing. |