MilliporeSigma
  • Home
  • Search Results
  • Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease.

Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease.

PloS one (2015-03-17)
Hila Zigdon, Alon Savidor, Yishai Levin, Anna Meshcheriakova, Raphael Schiffmann, Anthony H Futerman
ABSTRACT

Gaucher disease, a recessive inherited metabolic disorder caused by defects in the gene encoding glucosylceramidase (GlcCerase), can be divided into three subtypes according to the appearance of symptoms associated with central nervous system involvement. We now identify a protein, glycoprotein non-metastatic B (GPNMB), that acts as an authentic marker of brain pathology in neurological forms of Gaucher disease. Using three independent techniques, including quantitative global proteomic analysis of cerebrospinal fluid (CSF) in samples from Gaucher disease patients that display neurological symptoms, we demonstrate a correlation between the severity of symptoms and GPNMB levels. Moreover, GPNMB levels in the CSF correlate with disease severity in a mouse model of Gaucher disease. GPNMB was also elevated in brain samples from patients with type 2 and 3 Gaucher disease. Our data suggest that GPNMB can be used as a marker to quantify neuropathology in Gaucher disease patients and as a marker of treatment efficacy once suitable treatments towards the neurological symptoms of Gaucher disease become available.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Iodoacetamide, Single use vial of 56 mg
Sigma-Aldrich
Water-16O, ≥99.94 atom % 16O
Sigma-Aldrich
Iodoacetamide, BioUltra
Sigma-Aldrich
Sodium deoxycholate, ≥97% (titration)
Sigma-Aldrich
Iodoacetamide, ≥99% (NMR), crystalline
Sigma-Aldrich
Triton X-100, laboratory grade
Sigma-Aldrich
Sodium dodecyl sulfate solution, BioUltra, for molecular biology, 20% in H2O
Sigma-Aldrich
Sodium dodecyl sulfate solution, BioUltra, for molecular biology, 10% in H2O
Sigma-Aldrich
Sodium deoxycholate, BioXtra, ≥98.0% (dry matter, NT)
Sodium laurilsulfate, European Pharmacopoeia (EP) Reference Standard
SAFC
Sodium deoxycholate
SAFC
Iodoacetamide
Sigma-Aldrich
Water, deionized
Sigma-Aldrich
Water, ACS reagent
Sigma-Aldrich
Water, HPLC Plus
Sigma-Aldrich
Water, suitable for HPLC
Sigma-Aldrich
Sodium dodecyl sulfate, BioReagent, suitable for electrophoresis, for molecular biology, ≥98.5% (GC), free-flowing, Redi-Dri
Sigma-Aldrich
Sodium chloride solution, 0.85%
Sigma-Aldrich
Sodium dodecyl sulfate, ACS reagent, ≥99.0%
Sigma-Aldrich
Water, deuterium-depleted, ≤1 ppm (Deuterium oxide)
Sigma-Aldrich
Sodium chloride, AnhydroBeads, −10 mesh, 99.99% trace metals basis
Sigma-Aldrich
Sodium chloride, AnhydroBeads, −10 mesh, 99.999% trace metals basis
Sigma-Aldrich
Water, for molecular biology, sterile filtered
Sigma-Aldrich
Water, for cell biology, sterile ultrafiltered
Sigma-Aldrich
Sodium chloride-35Cl, 99 atom % 35Cl
Sigma-Aldrich
Sodium chloride, random crystals, optical grade, 99.9% trace metals basis
Sigma-Aldrich
Sodium chloride, 99.999% trace metals basis
Supelco
Water, for HPCE, for luminescence, suitable for UV/Vis spectroscopy
Sigma-Aldrich
Sodium dodecyl sulfate, ≥90%
Sigma-Aldrich
Sodium dodecyl sulfate, tested according to NF, mixture of sodium alkyl sulfates consisting mainly of sodium dodecyl sulfate