Bookmark and Share
BioAssay: AID 478974

Activation of purified acid alpha-glucosidase in human spleen homogenates using resorufin alpha-D-glucopyranoside as substrate at 10 uM after 45 mins relative to basal

Pompe disease is a lysosomal storage disease (LSD) caused by a deficiency in the lysosomal enzyme acid alpha-glucosidase. In several LSDs, enzyme inhibitors have been used as small molecule chaperones to facilitate and increase the translocation of mutant protein from the endoplasmic reticulum to the lysosome. Enzyme activators with chaperone activity would be even more desirable as they would more ..
_
   
 Tested Compounds
 Tested Compounds
All(1)
 
 
Unspecified(1)
 
 
 Tested Substances
 Tested Substances
All(1)
 
 
Unspecified(1)
 
 
 Related BioAssays
 Related BioAssays
AID: 478974
Data Source: ChEMBL (629837)
Depositor Category: Literature, Extracted
BioAssay Version:
Deposit Date: 2010-09-27
Modify Date: 2014-08-23

Data Table ( Complete ):           View All Data
Target
Sequence: RecName: Full=Lysosomal alpha-glucosidase; AltName: Full=Acid maltase; AltName: Full=Aglucosidase alfa; Contains: RecName: Full=76 kDa lysosomal alpha-glucosidase; Contains: RecName: Full=70 kDa lysosomal alpha-glucosidase; Flags: Precursor
Description ..   
Protein Family: maltase-glucoamylase, sucrase-isomaltase, lysosomal acid alpha-glucosidase
Comment ..   

Gene:GAA     Related Protein 3D Structures     More BioActivity Data..
Tested Compound:
Description:
Title: Evaluation of 2-thioxo-2,3,5,6,7,8-hexahydropyrimido[4,5-d]pyrimidin-4(1H)-one analogues as GAA activators.

Abstract: Pompe disease is a lysosomal storage disease (LSD) caused by a deficiency in the lysosomal enzyme acid alpha-glucosidase. In several LSDs, enzyme inhibitors have been used as small molecule chaperones to facilitate and increase the translocation of mutant protein from the endoplasmic reticulum to the lysosome. Enzyme activators with chaperone activity would be even more desirable as they would not inhibit the enzyme after translocation and might potentiate the activity of the enzyme that is successfully translocated. Herein we report our initial findings of a new series of acid alpha-glucosidase activators.
(PMID: 20206419)
Categorized Comment - additional comments and annotations
From BioAssay Depositor:
Assay Type: Binding
Target Type: Target is a single protein chain
Assay Data Source: Scientific Literature
Protein Target Class: enzyme hydrolase
Result Definitions
TIDNameDescriptionHistogramTypeUnit
OutcomeThe BioAssay activity outcomeOutcome
1Fold change activity commentFold change activity commentString
2Fold change standard flagFold change standard flagInteger
3Fold change qualifierFold change qualifierString
4Fold change published valueFold change published valueFloat
5Fold change standard valueFold change standard valueFloat

Data Table (Concise)
Data Table ( Complete ):     View All Data
Classification
PageFrom: