Recombinant Human Prickle-like protein 2 (PRICKLE2), partial
Description
General Information
Recombinant Human Prickle-like protein 2 (PRICKLE2), partial is a protein encoded by the PRICKLE2 gene in humans . PRICKLE2 is a homolog of the Drosophila prickle protein, which is involved in planar cell polarity (PCP) . The exact function of PRICKLE2 is not fully understood, but studies suggest it plays a role in seizure prevention and neuronal polarity . Mutations in the PRICKLE2 gene have been associated with progressive myoclonic epilepsy type 5 .
PRICKLE2 is located on chromosome 3 in humans and has several aliases, including EPM5 . It contains a Prickle-Espinas-Testin (PET) domain and LIM domains .
Biological Information
Function and Significance
PRICKLE2 is a core PCP protein that regulates axon number and the axon initial segment (AIS) . The PCP signaling mechanism involves two protein complexes that segregate to opposite sides of the cell . Prickle proteins, including PRICKLE2, are fundamental to this process .
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Neuronal Polarity: PRICKLE2 plays a critical role in establishing neuronal polarity .
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Axon Initial Segment (AIS): PRICKLE2 influences the AIS, a specialized region of the neuron crucial for initiating action potentials .
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Seizure Prevention: Mouse studies suggest a role in seizure prevention .
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Microtubule Organization: Prickle proteins control microtubule cytoskeleton organization in planar polarized cells .
4.1. Prickle2 and Neuronal Polarity
A study investigated the role of PRICKLE2 in neuronal polarity by examining the effects of PRICKLE2 depletion on neurons .
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Multiple-AIS/Axon Phenotype: Depletion of PRICKLE2 resulted in a significant proportion of neurons displaying more than three neurites immunoreactive for AnkG/PRICKLE2, indicating an alteration of neuronal polarity . Approximately two-thirds of PRICKLE2-depleted neurons exhibited this phenotype .
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Reduction in Protein Levels: The multiple-AIS/axon phenotype correlated with a substantial decrease in both PRICKLE2 and AnkG levels compared to the control group .
| Protein | Control Levels (Arbitrary Units) | shPrickle2A Levels (Arbitrary Units) | shPrickle2B Levels (Arbitrary Units) |
|---|---|---|---|
| Prickle2 | 10.8 ± 0.3 × 10^5 | 2.72 ± 0.1 × 10^5 | 4.32 ± 0.2 × 10^5 |
| AnkG | 15.5 ± 0.5 × 10^5 | 4.88 ± 0.2 × 10^5 | 9.78 ± 0.3 × 10^5 |
4.2. Electrophysiological Properties
The impact of PRICKLE2 depletion on the electrophysiological properties of neurons has been examined .
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Input Resistance: At DIV8 (days in vitro), neurons depleted of PRICKLE2 showed a significantly higher input resistance compared to the control group. A similar trend was seen at DIV22 .
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Maximum Firing Frequency: At DIV8, PRICKLE2-depleted neurons exhibited a lower maximum firing frequency compared to the control group .
| Property | DIV8 Control | DIV8 shPk2 | DIV22 Control | DIV22 shPk2 |
|---|---|---|---|---|
| Resting Membrane Potential | -50.1 ± 2.9 mV | -52.7 ± 2.5 mV | -59.9 ± 1 mV | -58.8 ± 0.8 mV |
| Input Resistance | 722 ± 104 megohms | 1406 ± 269 megohms | 183.6 ± 35.8 megohms | 283.7 ± 35.5 megohms |
| Firing Threshold | 25.6 ± 6 pA | 38 ± 5 pA | 198 ± 23 pA | 138 ± 22 pA |
| Slope F-I Curve | 0.29 ± 0.04 Hz/pA | 0.27 ± 0.06 Hz/pA | 62.6 ± 14 Hz/pA | 99.7 ± 25.6 Hz/pA |
| Maximum Firing Frequency | 7.4 ± 1.7 Hz | 3.3 ± 0.9 Hz | 19.2 ± 3.7 Hz | 16.0 ± 3.7 Hz |
Applications
Product Specs
Note: While we will prioritize shipping the format currently in stock, please specify any format requirements in your order notes for customized preparation.
Note: All proteins are shipped with standard blue ice packs. Dry ice shipping requires prior arrangement and incurs additional charges.
The tag type will be determined during production. If you require a specific tag type, please inform us; we will prioritize its development.
Target Background
- Two autism-spectrum-disorder patients presented with distinct, paternally inherited, heterozygous, non-synonymous PRICKLE2 variants (p.E8Q and p.V153I), also observed in their affected siblings. These variants demonstrated deficits in morphological and electrophysiological assays. PMID: 23711981
