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Rabbit Anti-Doppel/FITC Conjugated antibody
background:
Prion diseases or transmissible spongiform encephalopathies (TSEs) are manifested as genetic, infectious or sporadic, lethal neurodegenerative disorders involving alterations of the prion protein (PrP). Infectious PrPSc is highly expressed in the brain of animals affected by TSEs, including scrapie in sheep, BSE in cattle, and Cruetzfeldt-Jacob disease in humans. The PRND gene locus, located on human chromosome 20p, encodes for the doppel protein (Dpl), which exhibits approximately 25% sequence homology with PrP. Dpl is characterized by an alpha-helical conformation, intramolecular disulfide bonds, and two N-linked oligosaccharides, and it is presented on the cell surface by a glycosylphosphatidylinositol anchor. Dpl is highly expressed in adult testis and heart and is detectable in the brain of neonatal mice. Dpl does not appear to contribute to prion disease progression, but ectopic expression of Dpl is implicated in neuronal degeneration of ataxic PRP-deficient mice. Dpl is also thought to play a role in angiogenesis, specifically maturation of the blood-brain barrier.
Subcellular Location:
ell membrane; Lipid-anchor, GPI-anchor
Tissue Specificity:
Expressed in testis.
Similarity:
Belongs to the prion family.
Database links:
Entrez Gene: 23627 Human
Entrez Gene: 26434 Mouse
Entrez Gene: 113910 Rat
Omim: 604263 Human
SwissProt: Q9UKY0 Human
SwissProt: Q9QUG3 Mouse
Unigene: 406696 Human
Unigene: 36750 Mouse
Unigene: 94278 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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