| Id |
Subject |
Object |
Predicate |
Lexical cue |
| T1 |
98-106 |
DRI_Background |
denotes |
Neuronal |
| T2 |
129-291 |
DRI_Background |
denotes |
(NCLs) are a group of lysosomal storage disorders characterized pathologically by neuronal accumulation of autofluorescent storage material and neurodegeneration. |
| T3 |
292-419 |
DRI_Approach |
denotes |
An ovine NCL form is caused by a recessive point mutation in the cathepsin D gene, which encodes a lysosomal aspartyl protease. |
| T4 |
420-535 |
DRI_Outcome |
denotes |
This mutation results in typical NCL pathology with neurodegeneration and characteristic neuronal storage material. |
| T5 |
536-638 |
DRI_Outcome |
denotes |
We have generated a Drosophila NCL model by inactivating the conserved Drosophila cathepsin D homolog. |
| T6 |
639-717 |
DRI_Approach |
denotes |
We report here that cathepsin D mutant flies exhibit the key features of NCLs. |
| T7 |
718-877 |
DRI_Background |
denotes |
They show progressive neuronal accumulation of autofluorescent storage inclusions, which are also positive for periodic acid Schiff and luxol fast blue stains. |
| T8 |
878-1087 |
DRI_Background |
denotes |
Ultrastructurally, the storage material is composed of membrane-bound granular electron-dense material, similar to the granular osmiophilic deposits found in the human infantile and ovine congenital NCL forms. |
| T9 |
1171-1361 |
DRI_Outcome |
denotes |
Our results suggest that the metabolic pathway leading to NCL pathology is highly conserved during evolution, and that cathepsin D mutant flies can be used to study the pathogenesis of NCLs. |
