Anticorps Prion protein PrP

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Prion protein PrP est un gène codé par le symbole PRNP. Communément appelé aussi: Major prion protein; PrP; ASCR; PrP27-30; PrP33-35C; PRNP; ALTPRP; PRIP; PRP. Prion protein PrP a une masse de 27.66kDa, une longueur d'acide aminé de 253, et est impliqué dans les maladies: Creutzfeldt-Jakob disease; Fatal familial insomnia; Gerstmann-Straussler disease; Huntington disease-like 1; Kuru; Spongiform encephalopathy with neuropsychiatric features.

Nous proposons 14 des anticorps contre Prion protein PrP, élevé dans Lapin et Souris, qui sont appropriés pour le WB, IHC, ELISA et Cytométrie en Flux avec des échantillons dérivés de Humain, Souris, Rat, Bovin, Mouton et Hamster.

Informations sur les Gènes et les Protéines

Résumé UniProt
Its primary physiological function is unclear. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May promote myelin homeostasis through acting as an agonist for ADGRG6 receptor. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro) (By similarity). 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).
Résumé Entrez
The protein encoded by this gene is a membrane glycosylphosphatidylinositol-anchored glycoprotein that tends to aggregate into rod-like structures. The encoded protein contains a highly unstable region of five tandem octapeptide repeats. This gene is found on chromosome 20, approximately 20 kbp upstream of a gene which encodes a biochemically and structurally similar protein to the one encoded by this gene. Mutations in the repeat region as well as elsewhere in this gene have been associated with Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Straussler disease, Huntington disease-like 1, and kuru. An overlapping open reading frame has been found for this gene that encodes a smaller, structurally unrelated protein, AltPrp. Alternative splicing results in multiple transcript variants.
Implication dans la maladie
Creutzfeldt-Jakob disease: Occurs primarily as a sporadic disorder (1 per million), while 10-15% are familial. Accidental transmission of CJD to humans appears to be iatrogenic (contaminated human growth hormone (HGH), corneal transplantation, electroencephalographic electrode implantation, etc.). Epidemiologic studies have failed to implicate the ingestion of infected animal meat in the pathogenesis of CJD in human. The triad of microscopic features that characterize the prion diseases consists of (1) spongiform degeneration of neurons, (2) severe astrocytic gliosis that often appears to be out of proportion to the degree of nerve cell loss, and (3) amyloid plaque formation. CJD is characterized by progressive dementia and myoclonic seizures, affecting adults in mid-life. Some patients present sleep disorders, abnormalities of high cortical function, cerebellar and corticospinal disturbances. The disease ends in death after a 3-12 months illness.

Fatal familial insomnia: Autosomal dominant disorder and is characterized by neuronal degeneration limited to selected thalamic nuclei and progressive insomnia.

Gerstmann-Straussler disease: A rare inherited prion disease characterized by adult onset of memory loss, dementia, ataxia, and pathologic deposition of amyloid-like plaques in the brain. GSD presents with progressive limb and truncal ataxia, dysarthria, and cognitive decline in the thirties and forties, and the average disease duration is 7 years.

Huntington disease-like 1: Autosomal dominant, early-onset neurodegenerative disorder with prominent psychiatric features.

Kuru: Kuru is transmitted during ritualistic cannibalism, among natives of the New Guinea highlands. Patients exhibit various movement disorders like cerebellar abnormalities, rigidity of the limbs, and clonus. Emotional lability is present, and dementia is conspicuously absent. Death usually occurs from 3 to 12 month after onset.

Spongiform encephalopathy with neuropsychiatric features: Autosomal dominant presenile dementia with a rapidly progressive and protracted clinical course. The dementia was characterized clinically by frontotemporal features, including early personality changes. Some patients had memory loss, several showed aggressiveness, hyperorality and verbal stereotypy, others had parkinsonian symptoms.
Similitudes de séquence
Belongs to the prion family.
Modification post-traductionnelle
The glycosylation pattern (the amount of mono-, di- and non-glycosylated forms or glycoforms) seems to differ in normal and CJD prion.
Localisation cellulaire
Cell membrane. Golgi apparatus.

Targeted to lipid rafts via association with the heparan sulfate chains of GPC1. Colocates, in the presence of Cu(2+), to vesicles in para- and perinuclear regions, where both proteins undergo internalization. Heparin displaces PRNP from lipid rafts and promotes endocytosis.
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Western Blot - Anti-Prion protein PrP Antibody (A92782) - Antibodies.com
Voir le roduitKO Validé
Western Blot - Anti-Prion protein PrP Antibody [ARC1315] (A307424) - Antibodies.com
Western Blot - Anti-Prion protein PrP Antibody (A306421) - Antibodies.com
Western Blot - Anti-Prion Protein PrP Antibody [EM-20] (A85591) - Antibodies.com
Voir le roduitTaille d'Essai de 10µg
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Voir le roduitTaille d'Essai de 10µg
Western blot - PRNP Antibody from Signalway Antibody (32732) - Antibodies.com
(3)
Anti-PRNP Antibody from Bioworld Technology (BS7389) - Antibodies.com
(2)
PRNP Antibody from Signalway Antibody (43022) - Antibodies.com
(3)
Figure. Identification of Prion protein in crude cell extract by Western blotting using the monoclonal antibody 2C5-5. Lane 1: Extract of rabbit kidney cells RK13 over-expressing prion protein Lane 2: Negative control; extract of the vector infected cells Lane 3: Negative control; extract of RK13 cells
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