| Id |
Subject |
Object |
Predicate |
Lexical cue |
| T1 |
0-82 |
Sentence |
denotes |
Presence of an unusual GM2 derivative, taurine-conjugated GM2, in Tay-Sachs brain. |
| T2 |
83-162 |
Sentence |
denotes |
Tay-Sachs disease (TSD) is a classical glycosphingolipid (GSL) storage disease. |
| T3 |
163-360 |
Sentence |
denotes |
Although the genetic and biochemical bases for a massive cerebral accumulation of ganglioside GM2 in TSD have been well established, the mechanism for the neural dysfunction in TSD remains elusive. |
| T4 |
361-477 |
Sentence |
denotes |
Upon analysis of GSLs from a variant B TS brain, we have detected a novel GSL that has not been previously revealed. |
| T5 |
478-517 |
Sentence |
denotes |
We have isolated this GSL in pure form. |
| T6 |
518-757 |
Sentence |
denotes |
Using NMR spectroscopy, mass spectrometry, and chemical synthesis, the structure of this unusual GSL was established to be a taurine-conjugated GM2 (tauro-GM2) in which the carboxyl group of N-acetylneuraminic acid was amidated by taurine. |
| T7 |
758-923 |
Sentence |
denotes |
Using a rabbit anti-tauro-GM2 serum, we also detected the presence of tauro-GM2 in three other small brain samples from one variant B and two variant O TSD patients. |
| T8 |
924-1003 |
Sentence |
denotes |
On the other hand, tauro-GM2 was not found in three normal human brain samples. |
| T9 |
1004-1165 |
Sentence |
denotes |
The presence of tauro-GM2 in TS brains, but not in normal brains, indicates the possible association of this unusual GM2 derivative with the pathogenesis of TSD. |
| T10 |
1166-1293 |
Sentence |
denotes |
Our findings point to taurine conjugation as a heretofore unelucidated mechanism for TS brain to cope with water-insoluble GM2. |
