Id |
Subject |
Object |
Predicate |
Lexical cue |
TextSentencer_T1 |
0-95 |
Sentence |
denotes |
Functional and structural studies of wild type SOX9 and mutations causing campomelic dysplasia. |
TextSentencer_T2 |
96-195 |
Sentence |
denotes |
In humans, mutations in SOX9 result in a skeletal malformation syndrome, campomelic dysplasia (CD). |
TextSentencer_T3 |
196-261 |
Sentence |
denotes |
The present study investigated two major classes of CD mutations: |
TextSentencer_T4 |
262-397 |
Sentence |
denotes |
1) point mutations in the high mobility group (HMG) domain and 2) truncations and frameshifts that alter the C terminus of the protein. |
TextSentencer_T5 |
398-562 |
Sentence |
denotes |
We analyzed the effect of one novel mutation and three other point mutations in the HMG domain of SOX9 on the DNA binding and DNA bending properties of the protein. |
TextSentencer_T6 |
563-743 |
Sentence |
denotes |
The F12L mutant HMG domain shows negligible DNA binding, the H65Y mutant shows minimal DNA binding, whereas the A19V mutant shows near wild type DNA binding and bends DNA normally. |
TextSentencer_T7 |
744-840 |
Sentence |
denotes |
Interestingly, the P70R mutant has altered DNA binding specificity, but also bends DNA normally. |
TextSentencer_T8 |
841-940 |
Sentence |
denotes |
The effects of the point mutations were interpreted using a molecular model of the SOX9 HMG domain. |
TextSentencer_T9 |
941-1161 |
Sentence |
denotes |
We analyzed the effects upon transcription of mutations resembling the truncation and frameshift mutations in CD patients, and found that progressive deletion of the C terminus causes progressive loss of transactivation. |
TextSentencer_T10 |
1162-1346 |
Sentence |
denotes |
Maximal transactivation by SOX9 requires both the C-terminal domain rich in proline, glutamine, and serine and the adjacent domain composed entirely of proline, glutamine, and alanine. |
TextSentencer_T11 |
1347-1555 |
Sentence |
denotes |
Thus, CD arises by mutations that interfere with DNA binding by SOX9 or truncate the C-terminal transactivation domain and thereby impede the ability of SOX9 to activate target genes during organ development. |
T1 |
0-95 |
Sentence |
denotes |
Functional and structural studies of wild type SOX9 and mutations causing campomelic dysplasia. |
T2 |
96-195 |
Sentence |
denotes |
In humans, mutations in SOX9 result in a skeletal malformation syndrome, campomelic dysplasia (CD). |
T3 |
196-261 |
Sentence |
denotes |
The present study investigated two major classes of CD mutations: |
T4 |
262-397 |
Sentence |
denotes |
1) point mutations in the high mobility group (HMG) domain and 2) truncations and frameshifts that alter the C terminus of the protein. |
T5 |
398-562 |
Sentence |
denotes |
We analyzed the effect of one novel mutation and three other point mutations in the HMG domain of SOX9 on the DNA binding and DNA bending properties of the protein. |
T6 |
563-743 |
Sentence |
denotes |
The F12L mutant HMG domain shows negligible DNA binding, the H65Y mutant shows minimal DNA binding, whereas the A19V mutant shows near wild type DNA binding and bends DNA normally. |
T7 |
744-840 |
Sentence |
denotes |
Interestingly, the P70R mutant has altered DNA binding specificity, but also bends DNA normally. |
T8 |
841-940 |
Sentence |
denotes |
The effects of the point mutations were interpreted using a molecular model of the SOX9 HMG domain. |
T9 |
941-1161 |
Sentence |
denotes |
We analyzed the effects upon transcription of mutations resembling the truncation and frameshift mutations in CD patients, and found that progressive deletion of the C terminus causes progressive loss of transactivation. |
T10 |
1162-1346 |
Sentence |
denotes |
Maximal transactivation by SOX9 requires both the C-terminal domain rich in proline, glutamine, and serine and the adjacent domain composed entirely of proline, glutamine, and alanine. |
T11 |
1347-1555 |
Sentence |
denotes |
Thus, CD arises by mutations that interfere with DNA binding by SOX9 or truncate the C-terminal transactivation domain and thereby impede the ability of SOX9 to activate target genes during organ development. |