Detalhe da pesquisa
1.
Oral glycotoxins are a modifiable cause of dementia and the metabolic syndrome in mice and humans.
Proc Natl Acad Sci U S A
; 111(13): 4940-5, 2014 Apr 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-24567379
2.
Treatment with pentosan polysulphate in patients with MPS I: results from an open label, randomized, monocentric phase II study.
J Inherit Metab Dis
; 39(6): 831-837, 2016 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-27590017
3.
Intra-articular enzyme replacement therapy with rhIDUA is safe, well-tolerated, and reduces articular GAG storage in the canine model of mucopolysaccharidosis type I.
Mol Genet Metab
; 112(4): 286-93, 2014 Aug.
Artigo
em Inglês
| MEDLINE | ID: mdl-24951454
4.
The genetics of sphingolipid hydrolases and sphingolipid storage diseases.
Handb Exp Pharmacol
; (215): 3-32, 2013.
Artigo
em Inglês
| MEDLINE | ID: mdl-23579447
5.
Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses.
Proc Natl Acad Sci U S A
; 107(1): 222-7, 2010 Jan 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-20018674
6.
New paradigms for the treatment of lysosomal storage diseases: targeting the endocannabinoid system as a therapeutic strategy.
Orphanet J Rare Dis
; 16(1): 151, 2021 03 25.
Artigo
em Inglês
| MEDLINE | ID: mdl-33766102
7.
Acid Ceramidase Protects Against Hepatic Ischemia/Reperfusion Injury by Modulating Sphingolipid Metabolism and Reducing Inflammation and Oxidative Stress.
Front Cell Dev Biol
; 9: 633657, 2021.
Artigo
em Inglês
| MEDLINE | ID: mdl-34026750
8.
Identification and characterization of eight novel SMPD1 mutations causing types A and B Niemann-Pick disease.
Mol Med
; 16(7-8): 316-21, 2010.
Artigo
em Inglês
| MEDLINE | ID: mdl-20386867
9.
Growth Plate Pathology in the Mucopolysaccharidosis Type VI Rat Model-An Experimental and Computational Approach.
Diagnostics (Basel)
; 10(6)2020 May 31.
Artigo
em Inglês
| MEDLINE | ID: mdl-32486376
10.
Pentosan Polysulfate Treatment of Mucopolysaccharidosis Type IIIA Mice.
JIMD Rep
; 43: 37-52, 2019.
Artigo
em Inglês
| MEDLINE | ID: mdl-29654542
11.
Safety Study of Sodium Pentosan Polysulfate for Adult Patients with Mucopolysaccharidosis Type II.
Diagnostics (Basel)
; 9(4)2019 Dec 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-31861164
12.
Efficacy of pentosan polysulfate in in vitro models of lysosomal storage disorders: Fabry and Gaucher Disease.
PLoS One
; 14(5): e0217780, 2019.
Artigo
em Inglês
| MEDLINE | ID: mdl-31150494
13.
Enzyme replacement therapy for Farber disease: Proof-of-concept studies in cells and mice.
BBA Clin
; 7: 85-96, 2017 Jun.
Artigo
em Inglês
| MEDLINE | ID: mdl-28275553
14.
A New Fluorescent Method to Detect Sulfamidase Activity in Blood, Tissue Extracts and Dried Blood Spots
J. inborn errors metab. screen
; 9: e20200021, 2021. tab, graf
Artigo
em Inglês
|
LILACS-Express
| ID: biblio-1154708
15.
Pentosan Polysulfate: Oral Versus Subcutaneous Injection in Mucopolysaccharidosis Type I Dogs.
PLoS One
; 11(4): e0153136, 2016.
Artigo
em Inglês
| MEDLINE | ID: mdl-27064989
16.
Dose responsive effects of subcutaneous pentosan polysulfate injection in mucopolysaccharidosis type VI rats and comparison to oral treatment.
PLoS One
; 9(6): e100882, 2014.
Artigo
em Inglês
| MEDLINE | ID: mdl-24964042
17.
Two siblings with Niemann-Pick disease (NPD) type B: clinical findings and novel mutations of the acid sphingomyelinase gene.
Indian J Pediatr
; 80(2): 163-4, 2013 Feb.
Artigo
em Inglês
| MEDLINE | ID: mdl-22367733
18.
Structural, compositional, and biomechanical alterations of the lumbar spine in rats with mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome).
J Orthop Res
; 31(4): 621-31, 2013 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-23192728
19.
Pentosan polysulfate: a novel therapy for the mucopolysaccharidoses.
PLoS One
; 8(1): e54459, 2013.
Artigo
em Inglês
| MEDLINE | ID: mdl-23365668
20.
Acid ceramidase maintains the chondrogenic phenotype of expanded primary chondrocytes and improves the chondrogenic differentiation of bone marrow-derived mesenchymal stem cells.
PLoS One
; 8(4): e62715, 2013.
Artigo
em Inglês
| MEDLINE | ID: mdl-23638138