| + |
GLA | up-regulates quantity by expression 
chemical modification
|
1,3-dichloro-7-hydroxy-9,9-dimethyl-9H-acridin-2-one |
0.8 |
| Identifier |
Residue |
Sequence |
Organism |
Cell Line |
| SIGNOR-261334 |
|
|
in vitro |
|
| pmid |
sentence |
| 31996391 |
DDAOG is cleaved by -galactosidase ( Lindvall et al., 2009 ) in the Trpv1 cells to produce 7-hydroxy-9H(I,3-dichloro-9,9-dimethylacridin-2-one (DDAO). |
|
| Publications: |
1 |
Organism: |
In Vitro |
| + |
TFE3 | up-regulates quantity by expression 
transcriptional regulation
|
GLA |
0.2 |
| Identifier |
Residue |
Sequence |
Organism |
Cell Line |
| SIGNOR-276823 |
|
|
|
|
| pmid |
sentence |
| 24448649 |
Overexpression of TFE3 in ARPE-19 cells increased the mRNA abundance of 16 of the 17 genes tested, including those encoding several subunits of the v-ATPase (ATP6V0B1, ATP6V0D1, ATP6V0D2, and ATP6V1C1), lysosomal transmembrane proteins (CD63, CLCN7, CLCN3, LAMP1, and MCOLN1), and lysosomal hydrolases (GAA, GBA, GLA, CTSA, CTSD, CTSF, CTSS, and HEXA) (Fig. 5A). Western blotting confirmed the increase in several lysosomal proteins including LAMP1, RagC (encoded by RRAGC), cathepsin D (encoded by CTSD), and ATP6V1C1 in TFE3-overexpressing cells (fig. S5A). |
|
| Publications: |
1 |
| + |
migalastat | down-regulates activity 
chemical inhibition
|
GLA |
0.8 |
| Identifier |
Residue |
Sequence |
Organism |
Cell Line |
| SIGNOR-261076 |
|
|
Homo sapiens |
Lymphoblastic Leukemia Cell |
| pmid |
sentence |
| 10866822 |
1-Deoxygalactonojirimycin was the most potent inhibitor of a-Gal A with an IC50 value of 0.04mm. |
|
| Publications: |
1 |
Organism: |
Homo Sapiens |
| + |
TFEB | up-regulates quantity by expression
transcriptional regulation
|
GLA |
0.29 |
| Identifier |
Residue |
Sequence |
Organism |
Cell Line |
| SIGNOR-276539 |
|
|
|
|
| pmid |
sentence |
| 19556463 |
Under aberrant lysosomal storage conditions, TFEB translocated from the cytoplasm to the nucleus, resulting in the activation of its target genes.|Expression analysis of lysosomal genes after TFEB overexpression and silencing. Blue bars show the fold change of the mRNA levels of lysosomal genes in TFEB- versus pcDNA3-transfected cells. |
|
| Publications: |
1 |
3.0
GLA
- 
- 