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iCell DopaNeurons TMEM175, 01279

Investigate mechanisms of sporadic Parkinson’s disease with our panel of iCell® DopaNeurons TMEM175 mutants, featuring engineered risk-associated, protective, and isogenic corrected mutations.

  • Consistent culture - Derived from human induced pluripotent stem cells (iPSCs), iCell DopaNeurons provide a pure, reproducible system.
  • Confirmed phenotype – TMEM175 mutations present channel conductance phenotypes consistent with lysosome and Parkinson’s disease literature.
  • Isogenic controls – Derived from the same parent donor iPSCs to provide a TMEM175 mutation panel that includes isogenic controls.
  • Native characteristics - iCell DopaNeurons faithfully reflect primary human dopaminergic neuron morphology and function.
  • Simple implementation - Arriving fully differentiated, iCell DopaNeurons are ready to thaw and use, supporting scalable disease research.
Catalog # GDN01279
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  • 1 vial Cryopreserved iCell DopaNeurons, 01279
  • 100 ml iCell Neural Base Medium 1
  • 2 ml iCell Neural Supplement B
  • 1 ml iCell Nervous System Supplement
  • User’s Guide

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DopaNeurons Parkinson's Disease differentiated from human iPS cells, frozen

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$2,465.00

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  • 1 vial Cryopreserved iCell DopaNeurons, 01279
  • User’s Guide

genetic status

Quantity (Cells Per Vials)

Catalog #

Catalog #:

DopaNeurons Parkinson's Disease differentiated from human iPS cells, frozen

From
$2,365.00

Product Overview

Transmembrane Protein 175 (TMEM175) is a proton/potassium channel that modulates lysosomal pH and has emerged as a promising druggable target for sporadic Parkinson’s Disease (PD) and lysosomal storage diseases (LSDs). Mutations in TMEM175 modify lysosomal pH which are connected to altered cellular protein degradation capabilities and alpha-synuclein aggregation. Specifically, the TMEM175 heterozygous M393T (393M/T) mutation, which reduces channel conductance and acidifies the lysosome, is recognized as a highly prevalent but lowly penetrant risk-factors for sporadic PD. Conversely, the Q65P heterozygous (65Q/P) mutation increases TMEM175 conductance and is hypothesized to be protective.

To facilitate drug discovery research for Parkinson’s Disease and LSDs, we engineered a panel of isogenic TMEM175 mutant human iPSC-derived iCell DopaNeurons, including 393M/T, 393M/M, and 65Q/P variants. Together, this panel will enable deep investigation into TMEM175 biology, support translational drug discovery, and link channel physiology with cellular biology and PD disease pathology in a human-relevant in vitro system.

Note: 01279 donor iPSCs are from an apparently healthy normal (AHN) donor. This line was found to contain a heterozygous M393T mutation in TMEM175, which is a highly prevalent and low penetrant Parkinson’s Disease risk-associated mutation.

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Need Help With This Product?

Our specialists are here to help you find the best product for your application.

Call Us

(877) 320-6688

 

OR

Our regular business hours are 9:00am to 5:00pm Central Time (USA)

Performance Data

Highly Pure & Consistent Differentiation Across TMEM175 Lines

TMEM175 DopaNeurons are manufactured to the same purity and viability specifications as our industry-standard iCell DopaNeurons. Differentiation of TMEM175 mutation lines results in iCell DopaNeurons with similar purity and dopaminergic marker expression as our standard iCell DopaNeurons. Cells were stained for tyrosine hydroxylase (TH, magenta), FOXA2 (white), MAP2 (green), and nuclei (Hoechst, blue). Vials of 393T/T homozygous mutant iCell DopaNeurons are available by custom request.

TMEM175 Mutant iCell DopaNeurons Develop Healthy Neural Networks

iCell DopaNeuron TMEM175 mutants form active neural networks (synchronized bursting) when cultured and recorded on microelectrode array (MEA) plates (Axion Biosystems), demonstrating that these cells are highly functional iPSC-derived dopaminergic neurons. Vials of 393T/T homozygous mutant iCell DopaNeurons are available by custom request.

Expected Channel Phenotype Recorded by Lysosomal Patch Clamp

Lysosomal Patch Clamp of TMEM175 iCell DopaNeurons was performed at 14 days and current-voltage relationship was examined under unstimulated and DCPIB, a TMEM175 agonist, exposure. (A) Schematic of lysosomal patch clamp. (B) Representative current-voltage traces from iCell DopaNeurons TMEM175 393M/M with exposure to different concentrations of DCPIB. (C) TMEM175 current density analysis shows elevated baseline conductance for the 65Q/P line and equal or reduced conductance for 393M/T and 393T/T lines, respectively, compared to 393M/M. (D) These data agree with previously published data. The mutation did not affect responsiveness to DCPIB as shown by similar EC50 curves between the mutants. Vials of 393T/T homozygous mutant iCell DopaNeurons are available by custom request.

Experiments conducted by Axxam S.p.A.

Product Highlights

iCell DopaNeurons TMEM175, 01279 Highlights

  • Disease-relevant – First commercial human iPSC-derived dopaminergic neurons featuring risk-associated TMEM175 mutations along with isogenic controls.
  • Recapitulate Lysosomal Phenotype – Lysosomal patch clamp confirms TMEM175 iCell DopaNeurons phenotype matches published literature.
  • Ready-to-use – Simply thaw, plate, and culture. Ready to assay between 7-14 days (assay dependent).
  • Isogenic co-culture – Derived from the same donor iPSC as our iCell Microglia and iCell Astrocytes 2.0, enabling fully isogenic Parkinson's disease model co-culture.