Need Help With This Product?
Our specialists are here to help you find the best product for your application.
iCell DopaNeurons PD GBA N370S, 11344 and Isogenic Control
Human iPSC-derived dopaminergic neurons from a Caucasian male (age 60-69) with a Parkinson's disease phenotype (genotype: GBA (N370S)), differentiated from blood-derived iPSCs.
Products
Kits
- Cryopreserved iCell DopaNeurons PD GBA N370S, 11344
- 100 ml iCell Neural Base Medium 1
- 2 ml iCell Neural Supplement B
- 1 ml iCell Nervous System Supplement
- User’s Guide
genetic status
Quantity (Cells Per Vials)
Catalog #
Catalog #:
DopaNeurons Parkinson's Disease differentiated from human iPS cells, frozen
From
$1,195.00
Cells Only
- Cryopreserved iCell DopaNeurons PD GBA N370S, 11344
- User’s Guide
genetic status
Quantity (Cells Per Vials)
Catalog #
Catalog #:
DopaNeurons Parkinson's Disease differentiated from human iPS cells, frozen
From
$1,095.00
Product Overview
Mutations in the GBA gene are a common genetic risk factor for Parkinson’s disease (PD), occurring in > 5% of all cases. The GBA gene encodes glucocerebrosidase (GCase), a lysosomal enzyme that metabolizes glucocerebroside into ceramide and glucose. The GBA N370S mutation is associated with aberrant α-synuclein aggregation, reduced GCase activity, malfunction of the autophagy-lysosomal pathway, and endosomal stress.
iCell® DopaNeurons PD GBA N370S were derived from induced pluripotent stem (iPS) cells from the Parkinson's Progression Markers Initiative (PPMI). Sponsored by The Michael J. Fox Foundation, PPMI partnered with FUJIFILM Cellular Dynamics (FCDI) to produce a large bank of Parkinson’s donor-derived iPS cells, including lines harboring Parkinson’s-associated genetic mutations, such as GBA N370S. Each cell line is supported by PPMI donor clinical, imaging, genomics, and biological data. (www.ppmi-info.org)
To enable investigation of the functional consequences of the mutation within this patient-derived line, we used genetic engineering to generate a N370S/N mutation-corrected isogenic cell line from the same donor.
iCell DopaNeurons lines are differentiated into human midbrain floorplate dopaminergic (DA) neurons according to protocols licensed and adapted from the Lorenz Studer lab (Memorial Sloan Kettering) and industrialized at FCDI.
Benefits of iCell DopaNeurons PD GBA N370S and isogenic GBA N370S/N cell lines:
- Fully differentiated, >80% pure midbrain DA neurons derived from GBA N370S donor iPS cells
- Express midbrain dopaminergic neuron markers (e.g., Lmx1, FoxA2, and TH)
- Isogenic control GBA N370S/N mutation-corrected cell line is available.
- iCell DopaNeurons PD GBA N370S exhibit:
- Reduced GCase activity
- Altered dopamine metabolism
- Elevated alpha-synuclein protein accumulation
- Increased neuronal degeneration compared to the apparently healthy normal control iCell DopaNeurons
- Accessible and consistent model for discovery and toxicology research of Parkinson’s disease.
- View all Parkinson’s Disease iCell DopaNeurons cell lines
Components:
Our regular business hours are 9:00am to 5:00pm Central Time (USA)
Need Help With This Product?
Our specialists are here to help you find the best product for your application.
Our regular business hours are 9:00am to 5:00pm Central Time (USA)
Technical Docs
PROTOCOLS
Application Protocols
Immunofluorescent Labeling of iCell DopaNeurons
Application Protocols
Using Liposome-mediated Transfection for Gene Delivery - iCell DopaNeurons
USER DOCS
User's Guides
iCell DopaNeurons User's Guide
Datasheet
iCell DopaNeurons Data Sheet
Biosafety Document
iCell DopaNeurons GBA N370S/N Mutation-corrected Control Biosafety Document
Safety Data Sheet
iCell Neural Supplement B Safety Data Sheet
Safety Data Sheet
iCell Nervous System Supplement Safety Data Sheet
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet - Asia
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet
Safety Data Sheet
iCell Neural Base Medium 1 Safety Data Sheet
Performance Data
Highly Pure Populations of GBA and LRRK2 Patient-derived iCell DopaNeurons
Top) Immunocytochemistry images of GBA N370S and LRRK2 G2019S iCell DopaNeurons stained for FOXA2 and TH antibodies show consistent purity of the differentiated dopaminergic neurons at day 14 cultures. Bottom) Multiple batches of iCell DopaNeurons were analyzed by flow cytometry for TH, FOXA2, and MAP2 protein expression after 3 div. Consistent expression is observed across cell lines for mutant and normal dopaminergic neurons. Each dot represents one manufactured lot of iCell DopaNeurons.
Explore GCase Activity and Lysosomal Biology
A) GBA expression (RNAseq) was performed on multiple batches of Parkinson’s (GBA N370S and LRRK2 G2019S) and iCell DopaNeurons from an apparently healthy normal (AHN) at 14 div. Significantly lower GBA mRNA expression was observed in Parkinson’s disease patient iPSC-derived dopaminergic neurons. B) At 14 div, GBA, LRRK2 and AHN iCell DopaNeurons were harvest, lysed, and evaluated for GCase activity (relative fluorescence units; RFU) following 40-minute incubation with 4-Methylumbelliferyl β-glucophyranoside (10mM) and conduritol-b-epoxide (2mM). GCase activity was blunted in Parkinson’s iCell DopaNeurons compared to AHN (*P-value<0.05, ***P-value<0.001 one way ANOVA with Dunnett’s test for mean comparisons).
Interrogate Parkinson’s disease Mutations on Cell Metabolism and Bioenergetics
A) Cell density titration of AHN iCell DopaNeurons showed changes in OCR assay signal on Day 21 on the Seahorse XF Pro Analyzer. Recommended cell density is 125,000 cells/well. B) PD panel of iCell DopaNeurons were on Day 14 for basal respiration and spare capacity. GBA and LRRK2 iCell DopaNeurons showed lower metrics compared to AHN and SCNA A53T engineered neurons.
Screen for Alpha-Synuclein Aggregation in Patient-derived DopaNeurons
Left) Representative images of Thioflavin (Green) staining for aggregated α-synuclein at 22 div in Parkinson’s (GBA N370S and LRRK2 G2019S) and iCell DopaNeurons from an apparently healthy normal (AHN) donor following 24 incubation with α-synuclein oligomer (4 µM/ml). Right) Quantitation of Thioflavin staining after 24 and 48 hours following incubation with α-synuclein oligomer shows increased protein aggregation in mutant DopaNeurons (n = 4, One-way ANOVA with Dunnett’s multiple comparisons test) (C). Meso scale discovery (MSD) assay for α-synuclein accumulation showed increased α-synuclein in GBA, LRRK2, and SNCA DopaNeurons compared to AHN (n = 4, One-way ANOVA with Dunnett’s multiple comparisons test).
Explore Neural Network Activity Differences in GBA and LRRK2 iCell DopaNeurons.
Parkinson’s (GBA N370S and LRRK2 G2019S) and iCell DopaNeurons from an apparently healthy normal (AHN) donor were cultured in BrainPhys™ media and compared in different neuronal activity parameters. Representative Axion Maestro multielectrode array (MEA) raster plots show lower activity and network strength in Parkinson’s Disease mutant neurons compared to the AHN cells.
Applications
- Drug targeted screening
- Neurotoxicology
- Parkinson’s disease modeling
- Gaucher’s disease modeling
Product Highlights
Easy to implement
Shipped cryopreserved with optimized media. Simply thaw and use.
High throughput
Available in multiple unit sizes to support both scalable research and drug screening programs.
Consistent culture
80% tyrosine hydroxylase (TH)-positive dopaminergic neurons provides a robust and reproducible cell culture system.
Backed by clinical data
Generated in partnership with the PPMI and supported by patient clinical information. Visit PPMI for more information.
Biologically relevant
Derived from Parkinson’s Disease donor material and selected for common disease-associated gene mutations.
Publications
Citing of GBA and LRRK2 iCell DopaNeurons in Publications Publications and presentations featuring GBA and LLRK2 iCell DopaNeurons should reference the Parkinson's Progression Markers Initiative (PPMI) and the Golub Capital iPSC PPMI Sub-study (www.ppmi-info.org) as the original iPS cell line(s) source for the differentiated cells used in these experiments. Publications should acknowledge that the Parkinson's Progression Markers Initiative (PPMI) is a public-private partnership funded by The Michael J. Fox Foundation for Parkinson’s Research and other funding partners. Publications using these cells should be made available without charge to the research community through the PPMI website, when not prohibited by publication copyright terms and conditions. For assistance in uploading to the PPMI website, please contact: https:/www.ppmi-info.org/contact-us/. ()