Details for: PRNP

Gene ID: 5621

Symbol: PRNP

Ensembl ID: ENSG00000171867

Description: prion protein (Kanno blood group)

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: polychromatophilic erythroblast (CL0000550)
    Fold Change: 418.2063
    Cell Significance Index: -65.0500
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 261.0752
    Cell Significance Index: -66.2200
  • Cell Name: embryonic stem cell (CL0002322)
    Fold Change: 172.7647
    Cell Significance Index: -71.1700
  • Cell Name: smooth muscle fiber of ileum (CL1000278)
    Fold Change: 142.7595
    Cell Significance Index: -67.4000
  • Cell Name: peripheral blood mononuclear cell (CL2000001)
    Fold Change: 123.6039
    Cell Significance Index: -63.5800
  • Cell Name: orthochromatic erythroblast (CL0000552)
    Fold Change: 56.0523
    Cell Significance Index: -69.1100
  • Cell Name: CD8-positive, alpha-beta regulatory T cell (CL0000795)
    Fold Change: 24.6024
    Cell Significance Index: -75.5700
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 21.2926
    Cell Significance Index: -57.0400
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 17.6987
    Cell Significance Index: -69.8400
  • Cell Name: epidermal Langerhans cell (CL0002457)
    Fold Change: 11.1145
    Cell Significance Index: -24.3300
  • Cell Name: fibroblast of mammary gland (CL0002555)
    Fold Change: 3.1375
    Cell Significance Index: 89.9400
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 2.8763
    Cell Significance Index: 78.2900
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: 2.8387
    Cell Significance Index: 38.7300
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 2.6713
    Cell Significance Index: 342.4400
  • Cell Name: hippocampal pyramidal neuron (CL1001571)
    Fold Change: 2.3163
    Cell Significance Index: 66.1000
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 2.2745
    Cell Significance Index: 225.0000
  • Cell Name: cone retinal bipolar cell (CL0000752)
    Fold Change: 2.2458
    Cell Significance Index: 17.3100
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 2.1793
    Cell Significance Index: 437.1700
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 2.1643
    Cell Significance Index: 776.3100
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: 1.7450
    Cell Significance Index: 91.6200
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: 1.7155
    Cell Significance Index: 199.9200
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 1.5728
    Cell Significance Index: 215.9900
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: 1.5152
    Cell Significance Index: 101.8800
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 1.5082
    Cell Significance Index: 245.2900
  • Cell Name: Sertoli cell (CL0000216)
    Fold Change: 1.4879
    Cell Significance Index: 20.8700
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: 1.1809
    Cell Significance Index: 55.5000
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 1.1134
    Cell Significance Index: 143.8400
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 1.0985
    Cell Significance Index: 485.6800
  • Cell Name: CD4-positive, alpha-beta memory T cell, CD45RO-positive (CL0001204)
    Fold Change: 0.9963
    Cell Significance Index: 29.2600
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 0.8199
    Cell Significance Index: 89.1800
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.7016
    Cell Significance Index: 45.2700
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.5811
    Cell Significance Index: 71.4500
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: 0.4704
    Cell Significance Index: 29.6500
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.4219
    Cell Significance Index: 29.8400
  • Cell Name: retinal rod cell (CL0000604)
    Fold Change: 0.3658
    Cell Significance Index: 4.3600
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.3620
    Cell Significance Index: 197.6700
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 0.3370
    Cell Significance Index: 25.1200
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 0.2986
    Cell Significance Index: 20.6500
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.2898
    Cell Significance Index: 52.2400
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.2217
    Cell Significance Index: 44.0000
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: 0.1568
    Cell Significance Index: 9.6400
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.1253
    Cell Significance Index: 23.8500
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: 0.1067
    Cell Significance Index: 4.7200
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.0795
    Cell Significance Index: 2.2900
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 0.0457
    Cell Significance Index: 2.7500
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: 0.0332
    Cell Significance Index: 21.0900
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 0.0077
    Cell Significance Index: 0.2700
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: 0.0036
    Cell Significance Index: 6.8200
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: -0.0096
    Cell Significance Index: -6.6600
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: -0.0153
    Cell Significance Index: -2.6100
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0183
    Cell Significance Index: -11.4000
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0242
    Cell Significance Index: -17.9400
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: -0.0264
    Cell Significance Index: -19.9900
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: -0.0369
    Cell Significance Index: -0.8000
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0405
    Cell Significance Index: -74.6500
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0435
    Cell Significance Index: -66.9500
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0437
    Cell Significance Index: -4.4600
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0555
    Cell Significance Index: -75.5100
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: -0.0617
    Cell Significance Index: -45.2700
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0841
    Cell Significance Index: -47.4200
  • Cell Name: direct pathway medium spiny neuron (CL4023026)
    Fold Change: -0.0930
    Cell Significance Index: -3.5200
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.1048
    Cell Significance Index: -47.5700
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: -0.1145
    Cell Significance Index: -5.1900
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.1578
    Cell Significance Index: -33.2400
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: -0.1732
    Cell Significance Index: -20.4200
  • Cell Name: vascular lymphangioblast (CL0005022)
    Fold Change: -0.1995
    Cell Significance Index: -3.5300
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.2044
    Cell Significance Index: -58.8200
  • Cell Name: hippocampal interneuron (CL1001569)
    Fold Change: -0.2082
    Cell Significance Index: -2.7000
  • Cell Name: CD14-positive, CD16-negative classical monocyte (CL0002057)
    Fold Change: -0.2602
    Cell Significance Index: -4.8100
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: -0.2767
    Cell Significance Index: -21.2400
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.2802
    Cell Significance Index: -40.7300
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.2860
    Cell Significance Index: -32.7700
  • Cell Name: cerebellar granule cell (CL0001031)
    Fold Change: -0.3793
    Cell Significance Index: -6.5000
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: -0.3829
    Cell Significance Index: -19.8900
  • Cell Name: pancreatic endocrine cell (CL0008024)
    Fold Change: -0.3958
    Cell Significance Index: -45.1800
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: -0.4189
    Cell Significance Index: -7.0100
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: -0.4809
    Cell Significance Index: -13.4400
  • Cell Name: cortical interneuron (CL0008031)
    Fold Change: -0.5875
    Cell Significance Index: -14.0900
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.6627
    Cell Significance Index: -69.0000
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: -0.6740
    Cell Significance Index: -18.0300
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.7282
    Cell Significance Index: -57.6700
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: -0.8724
    Cell Significance Index: -40.6800
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: -0.8808
    Cell Significance Index: -23.5200
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.9653
    Cell Significance Index: -54.1700
  • Cell Name: transit amplifying cell of small intestine (CL0009012)
    Fold Change: -0.9940
    Cell Significance Index: -20.6200
  • Cell Name: Purkinje cell (CL0000121)
    Fold Change: -1.0349
    Cell Significance Index: -22.6600
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: -1.0614
    Cell Significance Index: -27.9100
  • Cell Name: pvalb GABAergic cortical interneuron (CL4023018)
    Fold Change: -1.0959
    Cell Significance Index: -23.2600
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -1.1082
    Cell Significance Index: -67.9400
  • Cell Name: lamp5 GABAergic cortical interneuron (CL4023011)
    Fold Change: -1.2205
    Cell Significance Index: -26.3700
  • Cell Name: stratified epithelial cell (CL0000079)
    Fold Change: -1.2354
    Cell Significance Index: -45.3500
  • Cell Name: corneal epithelial cell (CL0000575)
    Fold Change: -1.2546
    Cell Significance Index: -17.8600
  • Cell Name: sst GABAergic cortical interneuron (CL4023017)
    Fold Change: -1.2815
    Cell Significance Index: -25.3400
  • Cell Name: corticothalamic-projecting glutamatergic cortical neuron (CL4023013)
    Fold Change: -1.2933
    Cell Significance Index: -41.1900
  • Cell Name: hippocampal astrocyte (CL0002604)
    Fold Change: -1.3030
    Cell Significance Index: -18.2200
  • Cell Name: L6b glutamatergic cortical neuron (CL4023038)
    Fold Change: -1.3296
    Cell Significance Index: -43.5300
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: -1.3598
    Cell Significance Index: -28.9600
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: -1.3761
    Cell Significance Index: -47.8200
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: -1.3916
    Cell Significance Index: -44.5700
  • Cell Name: ON-bipolar cell (CL0000749)
    Fold Change: -1.4154
    Cell Significance Index: -15.9900

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics:** 1. **Glycoprotein structure:** The prion protein is a glycoprotein composed of a 108-125 amino acid sequence, which is rich in beta-sheet structures and has a unique N-terminal domain. 2. **Cellular localization:** The prion protein is expressed on the surface of cells, where it can interact with other proteins and ligands. 3. **Immune response:** The prion protein plays a role in regulating the immune response, including the activation of T cells and the suppression of amyloid-beta formation. 4. **Neurological functions:** The prion protein is involved in neuronal development, synaptic plasticity, and the regulation of calcium ion import across the plasma membrane. **Pathways and Functions:** 1. **Amyloid-beta binding:** The prion protein binds to amyloid-beta, which can regulate its formation and aggregation. 2. **Aspartic-type endopeptidase inhibitor activity:** The prion protein inhibits aspartic-type endopeptidases, which are involved in protein processing and degradation. 3. **ATP-dependent protein binding:** The prion protein binds to ATP-dependent proteins, which can regulate its activity and localization. 4. **Axon guidance:** The prion protein is involved in axon guidance and neuronal development. 5. **Calcium-mediated signaling:** The prion protein regulates calcium ion import across the plasma membrane, which can activate various signaling pathways. 6. **Cell cycle regulation:** The prion protein regulates the cell cycle, including the activation of cyclin-dependent kinases and the suppression of cyclin-dependent kinase inhibitors. 7. **Neurotransmitter regulation:** The prion protein regulates the release and uptake of neurotransmitters, including glutamate and GABA. **Clinical Significance:** 1. **Prion diseases:** Mutations in the PRNP gene have been associated with several neurodegenerative diseases, including prion diseases, scrapie, and Creutzfeldt-Jakob disease. 2. **Amyloid-beta regulation:** The prion protein plays a role in regulating amyloid-beta formation and aggregation, which can contribute to neurodegenerative diseases. 3. **Immune dysfunction:** The prion protein is involved in regulating the immune response, including the activation of T cells and the suppression of amyloid-beta formation. 4. **Cancer:** The prion protein is overexpressed in various types of cancer, including prostate cancer and breast cancer, and may contribute to tumor progression. 5. **Neurological disorders:** The prion protein is involved in various neurological disorders, including Alzheimer's disease, Parkinson's disease, and Huntington's disease. In conclusion, the prion protein gene is a complex and multifaceted molecule that plays a crucial role in various biological processes, including immune response, neuronal development, and tissue homeostasis. Mutations in the PRNP gene have been associated with several neurodegenerative diseases, and understanding the functions and regulation of the prion protein is essential for the development of novel therapeutic strategies for these diseases.

Genular Protein ID: 705335050

Symbol: APRIO_HUMAN

Name: Alternative prion protein

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 11780052

Title: The DNA sequence and comparative analysis of human chromosome 20.

PubMed ID: 11780052

DOI: 10.1038/414865a

PubMed ID: 21478263

Title: An overlapping reading frame in the PRNP gene encodes a novel polypeptide distinct from the prion protein.

PubMed ID: 21478263

DOI: 10.1096/fj.10-173815

Sequence Information:

  • Length: 73
  • Mass: 8691
  • Checksum: 4360C28341A4CA52
  • Sequence:
  • MEHWGQPIPG AGQPWRQPLP TSGRWWLGAA SWWWLGAASW WWLGAAPWWW LGTASWWWLG 
    SRRWHPQSVE QAE

Genular Protein ID: 44896081

Symbol: PRIO_HUMAN

Name: Major prion protein

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 3755672

Title: Molecular cloning of a human prion protein cDNA.

PubMed ID: 3755672

DOI: 10.1089/dna.1986.5.315

PubMed ID: 1678248

Title: Genomic structure of the human prion protein gene.

PubMed ID: 1678248

PubMed ID: 9799790

Title: Complete genomic sequence and analysis of the prion protein gene region from three mammalian species.

PubMed ID: 9799790

DOI: 10.1101/gr.8.10.1022

PubMed ID: 10581485

Title: Novel PRNP sequence variant associated with familial encephalopathy.

PubMed ID: 10581485

DOI: 10.1002/(sici)1096-8628(19991215)88:6<653::aid-ajmg14>3.0.co;2-e

PubMed ID: 11780052

Title: The DNA sequence and comparative analysis of human chromosome 20.

PubMed ID: 11780052

DOI: 10.1038/414865a

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 3014653

Title: Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications.

PubMed ID: 3014653

DOI: 10.1126/science.3014653

PubMed ID: 1363802

Title: Deletion in the prion protein gene in a demented patient.

PubMed ID: 1363802

DOI: 10.1093/hmg/1.6.443

PubMed ID: 9384372

Title: A prion-linked psychiatric disorder.

PubMed ID: 9384372

DOI: 10.1038/36757

PubMed ID: 7485229

Title: SSCP analysis and sequencing of the human prion protein gene (PRNP) detects two different 24 bp deletions in an atypical Alzheimer's disease family.

PubMed ID: 7485229

DOI: 10.1002/ajmg.1320600104

PubMed ID: 1672107

Title: Amyloid protein of Gerstmann-Straussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58.

PubMed ID: 1672107

DOI: 10.1002/j.1460-2075.1991.tb07977.x

PubMed ID: 1683708

Title: Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene.

PubMed ID: 1683708

DOI: 10.1073/pnas.88.23.10926

PubMed ID: 9792871

Title: A Huntington disease-like neurodegenerative disorder maps to chromosome 20p.

PubMed ID: 9792871

DOI: 10.1086/302093

PubMed ID: 12732622

Title: Prion, amyloid beta-derived Cu(II) ions, or free Zn(II) ions support S-nitroso-dependent autocleavage of glypican-1 heparan sulfate.

PubMed ID: 12732622

DOI: 10.1074/jbc.m300394200

PubMed ID: 12214108

Title: The Thr183Ala mutation, not the loss of the first glycosylation site, alters the physical properties of the prion protein.

PubMed ID: 12214108

DOI: 10.3233/jad-2000-2104

PubMed ID: 16144413

Title: The octarepeat domain of the prion protein binds Cu(II) with three distinct coordination modes at pH 7.4.

PubMed ID: 16144413

DOI: 10.1021/ja053254z

PubMed ID: 18034490

Title: The prion protein is a combined zinc and copper binding protein: Zn2+ alters the distribution of Cu2+ coordination modes.

PubMed ID: 18034490

DOI: 10.1021/ja077146j

PubMed ID: 19059915

Title: Biosynthesis of prion protein nucleocytoplasmic isoforms by alternative initiation of translation.

PubMed ID: 19059915

DOI: 10.1074/jbc.m804051200

PubMed ID: 29222195

Title: Biosynthesis of prion protein nucleocytoplasmic isoforms by alternative initiation of translation.

PubMed ID: 29222195

DOI: 10.1074/jbc.w117.000658

PubMed ID: 19349973

Title: Mass-spectrometric identification and relative quantification of N-linked cell surface glycoproteins.

PubMed ID: 19349973

DOI: 10.1038/nbt.1532

PubMed ID: 19936054

Title: Glypican-1 mediates both prion protein lipid raft association and disease isoform formation.

PubMed ID: 19936054

DOI: 10.1371/journal.ppat.1000666

PubMed ID: 19381258

Title: Early onset prion disease from octarepeat expansion correlates with copper or zinc binding properties.

PubMed ID: 19381258

DOI: 10.1371/journal.ppat.1000390

PubMed ID: 20375014

Title: Prion fibrillization is mediated by a native structural element that comprises helices H2 and H3.

PubMed ID: 20375014

DOI: 10.1074/jbc.m110.111815

PubMed ID: 20564047

Title: Copper (II) promotes the formation of soluble neurotoxic PrP oligomers in acidic environment.

PubMed ID: 20564047

DOI: 10.1002/jcb.22743

PubMed ID: 21478263

Title: An overlapping reading frame in the PRNP gene encodes a novel polypeptide distinct from the prion protein.

PubMed ID: 21478263

DOI: 10.1096/fj.10-173815

PubMed ID: 21153684

Title: Characterizing the novel protein p33MONOX.

PubMed ID: 21153684

DOI: 10.1007/s11010-010-0690-4

PubMed ID: 10954699

Title: Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseases.

PubMed ID: 10954699

DOI: 10.1074/jbc.c000483200

PubMed ID: 10618385

Title: NMR solution structure of the human prion protein.

PubMed ID: 10618385

DOI: 10.1073/pnas.97.1.145

PubMed ID: 10900000

Title: NMR structures of three single-residue variants of the human prion protein.

PubMed ID: 10900000

DOI: 10.1073/pnas.97.15.8340

PubMed ID: 11524679

Title: Crystal structure of the human prion protein reveals a mechanism for oligomerization.

PubMed ID: 11524679

DOI: 10.1038/nsb0901-770

PubMed ID: 11900542

Title: Molecular features of the copper binding sites in the octarepeat domain of the prion protein.

PubMed ID: 11900542

DOI: 10.1021/bi011922x

PubMed ID: 14623188

Title: The octapeptide repeats in mammalian prion protein constitute a pH-dependent folding and aggregation site.

PubMed ID: 14623188

DOI: 10.1016/j.jmb.2003.09.048

PubMed ID: 8364585

Title: Mutations and polymorphisms in the prion protein gene.

PubMed ID: 8364585

DOI: 10.1002/humu.1380020303

PubMed ID: 8105771

Title: Genetic and infectious prion diseases.

PubMed ID: 8105771

DOI: 10.1001/archneur.1993.00540110011002

PubMed ID: 17468747

Title: Atomic structures of amyloid cross-beta spines reveal varied steric zippers.

PubMed ID: 17468747

DOI: 10.1038/nature05695

PubMed ID: 19204296

Title: Crystal structure of human prion protein bound to a therapeutic antibody.

PubMed ID: 19204296

DOI: 10.1073/pnas.0809170106

PubMed ID: 19927125

Title: Conformational diversity in prion protein variants influences intermolecular beta-sheet formation.

PubMed ID: 19927125

DOI: 10.1038/emboj.2009.333

PubMed ID: 2564168

Title: Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome.

PubMed ID: 2564168

DOI: 10.1038/338342a0

PubMed ID: 2783132

Title: Pro-->Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler syndrome.

PubMed ID: 2783132

DOI: 10.1016/0006-291x(89)92317-6

PubMed ID: 1347910

Title: Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178.

PubMed ID: 1347910

DOI: 10.1212/wnl.42.3.669

PubMed ID: 1671440

Title: New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindred.

PubMed ID: 1671440

DOI: 10.1016/0140-6736(91)91198-4

PubMed ID: 1975028

Title: Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia.

PubMed ID: 1975028

DOI: 10.1016/0140-6736(90)92073-q

PubMed ID: 1363810

Title: Mutant prion proteins in Gerstmann-Straussler-Scheinker disease with neurofibrillary tangles.

PubMed ID: 1363810

DOI: 10.1038/ng0492-68

PubMed ID: 1439789

Title: Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism.

PubMed ID: 1439789

DOI: 10.1126/science.1439789

PubMed ID: 8461023

Title: Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome.

PubMed ID: 8461023

DOI: 10.1006/bbrc.1993.1275

PubMed ID: 7902693

Title: A new point mutation of the prion protein gene in Creutzfeldt-Jakob disease.

PubMed ID: 7902693

DOI: 10.1002/ana.410340608

PubMed ID: 7902972

Title: A missense mutation at codon 105 with codon 129 polymorphism of the prion protein gene in a new variant of Gerstmann-Straussler-Scheinker disease.

PubMed ID: 7902972

DOI: 10.1212/wnl.43.12.2723-a

PubMed ID: 7699395

Title: A variant of Gerstmann-Straussler-Scheinker disease carrying codon 105 mutation with codon 129 polymorphism of the prion protein gene: a clinicopathological study.

PubMed ID: 7699395

DOI: 10.1016/0022-510x(94)90138-4

PubMed ID: 7906019

Title: Japanese family with Creutzfeldt-Jakob disease with codon 200 point mutation of the prion protein gene.

PubMed ID: 7906019

DOI: 10.1212/wnl.44.2.299

PubMed ID: 7913755

Title: Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease.

PubMed ID: 7913755

DOI: 10.1098/rstb.1994.0033

PubMed ID: 7783876

Title: Gerstmann-Straussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients.

PubMed ID: 7783876

DOI: 10.1212/wnl.45.6.1127

PubMed ID: 8797472

Title: Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Straussler-Scheinker disease (PrP-P102L mutation).

PubMed ID: 8797472

DOI: 10.1212/wnl.47.3.734

PubMed ID: 8909447

Title: Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease.

PubMed ID: 8909447

DOI: 10.1212/wnl.47.5.1305

PubMed ID: 9266722

Title: Familial spongiform encephalopathy associated with a novel prion protein gene mutation.

PubMed ID: 9266722

DOI: 10.1002/ana.410420203

PubMed ID: 9786248

Title: Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity.

PubMed ID: 9786248

DOI: 10.1097/00005072-199810000-00010

PubMed ID: 9482303

Title: Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease.

PubMed ID: 9482303

DOI: 10.1016/s0140-6736(05)78358-6

PubMed ID: 10987652

Title: Molecular genetics of human prion diseases in Germany.

PubMed ID: 10987652

DOI: 10.1007/s004399900124

PubMed ID: 10631141

Title: High prevalence of pathogenic mutations in patients with early-onset dementia detected by sequence analyses of four different genes.

PubMed ID: 10631141

DOI: 10.1086/302702

PubMed ID: 10790216

Title: Identification of three novel mutations (E196K, V203I, E211Q) in the prion protein gene (PRNP) in inherited prion diseases with Creutzfeldt-Jakob disease phenotype.

PubMed ID: 10790216

DOI: 10.1002/(sici)1098-1004(200005)15:5<482::aid-humu16>3.0.co;2-1

PubMed ID: 11709001

Title: A new PRNP mutation (G131V) associated with Gerstmann-Straussler-Scheinker disease.

PubMed ID: 11709001

DOI: 10.1001/archneur.58.11.1899

PubMed ID: 12690204

Title: Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics.

PubMed ID: 12690204

DOI: 10.1126/science.1083320

PubMed ID: 19923577

Title: A novel protective prion protein variant that colocalizes with kuru exposure.

PubMed ID: 19923577

DOI: 10.1056/nejmoa0809716

PubMed ID: 26061765

Title: A naturally occurring variant of the human prion protein completely prevents prion disease.

PubMed ID: 26061765

DOI: 10.1038/nature14510

Sequence Information:

  • Length: 253
  • Mass: 27661
  • Checksum: 43DB596BAAA66484
  • Sequence:
  • MANLGCWMLV LFVATWSDLG LCKKRPKPGG WNTGGSRYPG QGSPGGNRYP PQGGGGWGQP 
    HGGGWGQPHG GGWGQPHGGG WGQPHGGGWG QGGGTHSQWN KPSKPKTNMK HMAGAAAAGA 
    VVGGLGGYML GSAMSRPIIH FGSDYEDRYY RENMHRYPNQ VYYRPMDEYS NQNNFVHDCV 
    NITIKQHTVT TTTKGENFTE TDVKMMERVV EQMCITQYER ESQAYYQRGS SMVLFSSPPV 
    ILLISFLIFL IVG

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.