PRNP

PRNP (PRioN Protein) is the human gene encoding for the major prion protein PrP (for prion protein or protease-resistant protein), also known as CD230 (cluster of differentiation 230). Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body.

The protein can exist in multiple isoforms, the normal PrP^C, the disease-causing PrP^Sc, and an isoform located in mitochondria. The misfolded version PrP^Sc is associated with a variety of cognitive disorders and neurodegenerative diseases such as bovine spongiform encephalopathy, chronic wasting disease, Creutzfeldt–Jakob disease, fatal familial insomnia, feline spongiform encephalopathy, Gerstmann–Sträussler–Scheinker syndrome, kuru, scrapie, transmissible mink encephalopathy, ungulate spongiform encephalopathy, and variant Creutzfeldt-Jakob disease.

Gene

The human PRNP gene is located on the short (p) arm of chromosome 20 between the end (terminus) of the arm and position 12, from base pair 4,615,068 to base pair 4,630,233.

PRP

PRP may refer to:

Places

Medicine and biology

Mathematics and science

Organizations

Prp8

Prp8 refers to both the Prp8 protein and Prp8 gene. Prp8's name originates from its involvement in pre-mRNA processing. The Prp8 protein is a large, highly conserved, and unique protein that resides in the catalytic core of the spliceosome and has been found to have a central role in molecular rearrangements that occur there. Prp8 protein is a major central component of the catalytic core in the spliceosome, and the spliceosome is responsible for splicing of precursor mRNA that contains introns and exons. Unexpressed introns are removed by the spliceosome complex in order to create a more concise mRNA transcript. Splicing is just one of many different post-transcriptional modifications that mRNA must undergo before translation. Prp8 has also been hypothesized to be a cofactor in RNA catalysis.

History

The systematic name for the PRP8 protein is YHR165C. Prp8 protein is coded by a single gene in humans with 42 exons. The size of Prp8 ranges between 230-280 kDa depending on the organism. The sequence coding for the Prp8 protein is highly conserved between eukaryotic organisms, with a 61% identity match between humans and yeast in amino acid sequence. The Prp8 gene is located on chromosome VIII in yeast and chromosome 17 in humans.