Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:4.2.2.7 (
heparinase
)
1,270
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Aim:
Accumulation of heparan sulfate (HS) is associated with the neurodegenerative disorder
Mucopolysaccharidosis type IIIA
(
MPS IIIA
). Here, we compare HS levels in brain and cerebrospinal fluid (CSF) of
MPS IIIA
mice after treatment with a chemically modified sulfamidase (CM-rhSulfamidase).
Materials & methods:
Two LC-MS/MS methods were adapted from literature methodology, one to measure HS metabolites (HS
met
), the other to measure digests of HS after
heparinase
treatment (HS
dig
).
Results:
The HS
met
and HS
dig
methods showed similar relative reduction of HS in brain after CM-rhSulfamidase administration to
MPS IIIA
mice and the reduction was reflected also in CSF.
Conclusion:
The results of the two methods correlated and therefore the HS
dig
method can be used in clinical studies to determine HS levels in CSF from patients with
MPS IIIA
.
...
PMID:Robust LC-MS/MS methods for analysis of heparan sulfate levels in CSF and brain for application in studies of MPS IIIA. 3149 Jan 6
