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NameMajor prion protein
Synonyms
  • ALTPRP
  • ASCR
  • PRIP
  • PRP
  • PrP27-30
  • PrP33-35C
Gene NamePRNP
OrganismHuman
Amino acid sequence
>lcl|BSEQ0002199|Major prion protein
MANLGCWMLVLFVATWSDLGLCKKRPKPGGWNTGGSRYPGQGSPGGNRYPPQGGGGWGQP
HGGGWGQPHGGGWGQPHGGGWGQPHGGGWGQGGGTHSQWNKPSKPKTNMKHMAGAAAAGA
VVGGLGGYMLGSAMSRPIIHFGSDYEDRYYRENMHRYPNQVYYRPMDEYSNQNNFVHDCV
NITIKQHTVTTTTKGENFTETDVKMMERVVEQMCITQYERESQAYYQRGSSMVLFSSPPV
ILLISFLIFLIVG
Number of residues253
Molecular Weight27661.21
Theoretical pI9.23
GO Classification
Functions
  • microtubule binding
  • identical protein binding
  • tubulin binding
  • copper ion binding
Processes
  • regulation of potassium ion transmembrane transport
  • response to oxidative stress
  • cell cycle arrest
  • regulation of protein localization
  • negative regulation of interleukin-17 production
  • negative regulation of protein phosphorylation
  • axon guidance
  • metabolic process
  • negative regulation of interleukin-2 production
  • negative regulation of apoptotic process
  • cellular copper ion homeostasis
  • cellular response to copper ion
  • cellular response to drug
  • response to cadmium ion
  • negative regulation of T cell receptor signaling pathway
  • protein homooligomerization
  • negative regulation of interferon-gamma production
  • negative regulation of sequence-specific DNA binding transcription factor activity
  • negative regulation of activated T cell proliferation
  • negative regulation of calcineurin-NFAT signaling cascade
  • learning or memory
Components
  • extrinsic component of membrane
  • plasma membrane
  • cell surface
  • Golgi apparatus
  • extracellular exosome
  • membrane raft
  • cytoplasm
  • anchored component of membrane
  • nucleus
  • endoplasmic reticulum
General FunctionTubulin binding
Specific FunctionIts primary physiological function is unclear. Has cytoprotective activity against internal or environmental stresses. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro) (PubMed:12732622, PubMed:19936054, PubMed:20564047). Association with GPC1 (via its heparan sulfate chains) targets PRNP to lipid rafts. Also provides Cu(2+) or ZN(2+) for the ascorbate-mediated GPC1 deaminase degradation of its heparan sulfate side chains (By similarity).
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID190468
UniProtKB IDP04156
UniProtKB Entry NamePRIO_HUMAN
Cellular LocationCell membrane
Gene sequence
>lcl|BSEQ0019060|Major prion protein (PRNP)
ATGGCGAACCTTGGCTGCTGGATGCTGGTTCTCTTTGTGGCCACATGGAGTGACCTGGGC
CTCTGCAAGAAGCGCCCGAAGCCTGGAGGATGGAACACTGGGGGCAGCCGATACCCGGGG
CAGGGCAGCCCTGGAGGCAACCGCTACCCACCTCAGGGCGGTGGTGGCTGGGGGCAGCCT
CATGGTGGTGGCTGGGGGCAGCCTCATGGTGGTGGCTGGGGGCAGCCCCATGGTGGTGGC
TGGGGACAGCCTCATGGTGGTGGCTGGGGTCAAGGAGGTGGCACCCACAGTCAGTGGAAC
AAGCCGAGTAAGCCAAAAACCAACATGAAGCACATGGCTGGTGCTGCAGCAGCTGGGGCA
GTGGTGGGGGGCCTTGGCGGCTACATGCTGGGAAGTGCCATGAGCAGGCCCATCATACAT
TTCGGCAGTGACTATGAGGACCGTTACTATCGTGAAAACATGCACCGTTACCCCAACCAA
GTGTACTACAGGCCCATGGATGAGTACAGCAACCAGAACAACTTTGTGCACGACTGCGTC
AATATCACAATCAAGCAGCACACGGTCACCACAACCACCAAGGGGGAGAACTTCACCGAG
ACCGACGTTAAGATGATGGAGCGCGTGGTTGAGCAGATGTGTATCACCCAGTACGAGAGG
GAATCTCAGGCCTATTACCAGAGAGGATCGAGCATGGTCCTCTTCTCCTCTCCACCTGTG
ATCCTCCTGATCTCTTTCCTCATCTTCCTGATAGTGGGATGA
GenBank Gene IDM13899
GeneCard IDNot Available
GenAtlas IDPRNP
HGNC IDHGNC:9449
Chromosome Location20
Locus20p13
References
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