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Details on Person Periaxin (PRX) is a scaffolding protein that is part of a d...
| Class:Id | Summation:9619654 |
|---|---|
| _displayName | Periaxin (PRX) is a scaffolding protein that is part of a d... |
| _timestamp | 2018-09-18 13:50:44 |
| created | [InstanceEdit:9619650] Rothfels, Karen, 2018-09-18 |
| text | Periaxin (PRX) is a scaffolding protein that is part of a dystrophin:dystroglycan complex required for maintenance of the myelin sheath in Schwann cells. PRX homodimers interact with DRP2 to form a complex with dystroglycan at the basal lamina, anchoring the complex in the plasma membrane (Sherman et al, 2001; Han and Kursala, 2014). Mutations in PRX are associated with severe demyelinating peripheral neuropathies (Boerkoel et al, 2001; Guilbot et al, 2001). Expression of PRX initiates earlier than EGR2 during peripheral nerve cell myelination, indicating an EGR2-independent mechanism early during myelination (Parkinson et al, 2003). Candidate regulators of this early expression include SOX10 and EGR1, which is expressed in embryonic Schwann cells and with EGR2 at postnatal day 1 (Topilko et al, 1997). Sustained, upregulated expression of PRX during myelination depends on the binding of EGR2 and SOX10 to a binding site within the first intron, 4.5 kb from the transcription start site (Jones et al, 2007; Srinivasan et al, 2012). Consistent with this, expression of PRX is decreased in EGR2 null mice (Nagarajan et al, 2001; Boerkoel et al, 2001). |
| (summation) | [Reaction:9619657] EGR2 and SOX10 bind the PRX gene [Homo sapiens] |
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