| PRND Protein — Prion-Like Protein Doppel | |
|---|---|
| Protein Name | Prion-Like Protein Doppel (Dpl) |
| Gene | PRND |
| UniProt ID | Q9NSY5 |
| Molecular Weight | ~17 kDa (176 aa) |
| Subcellular Localization | Cell membrane (GPI-anchored) |
| Protein Family | Prion protein family |
| Expression | Testis, brain, heart, skeletal muscle |
PRND Protein (also known as Doppel or Dpl) is a prion protein family member encoded by the PRND gene on chromosome 20p13 [1]. The 176-amino acid GPI-anchored protein shares structural homology with the cellular prion protein (PrP^C) encoded by PRNP [2].
Doppel was discovered as an overexpressed protein in mice lacking the prion protein gene (Prnp^0/0), where it caused cerebellar ataxia and Purkinje cell degeneration [2]. This finding demonstrated the neurotoxic potential of the Doppel protein when misexpressed.
The Doppel protein contains:
Doppel shares structural features with PrP:
The normal function of Doppel is not fully understood:
PRND belongs to the prion protein gene family:
| Gene | Protein | Expression | Function |
|---|---|---|---|
| PRNP | PrP^C | Ubiquitous | Neuroprotection, synaptic function |
| PRND | Dpl | Testis > Brain | Less characterized |
| PRNT | - | Testis | Testis-specific |
| SPRN | Shadoo | Brain | Neuroprotection |
When overexpressed or misfolded, Doppel becomes neurotoxic:
While not a primary cause of human prion diseases:
Moore RC, et al. (2001). The prion-like protein Doppel is neurotoxic. Nature, 413(6855):522-526.
Silverman GL, et al. (pel is localized to the myelin sheath2000). Dop. Journal of Neuroscience, 20(17):6365-6374.
Watts JC, et al. (2009). Prion protein biology and disease. Current Topics in Microbiology and Immunology, 337:1-33.
Westaway D, et al. (2002). Targeting the prion protein. Progress in Brain Research, 138:133-147.