货号：AB5058

品牌：Merck Millipore

规格：200UL

目录价：￥5053.00

市场价格：￥4295.05

会员价格：￥4042.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Prion Protein Antibody, N-terminus, a.a. 78-97 | AB5058 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Specific for human prion protein (PrP). This antibody immunolabels amyloid plaques in formalin-fixed paraffin sections from Creutzfeld-Jakob Disease (CJD) brain.The prion protein is a large membrane protein that occurs normally in neurons of the human brain and is thought to be involved in synaptic transmission. In prion diseases, such as CJD, Gerstmann-Straussler-Scheinker syndrome (GSS), Fatal Familial Insomnia (FFI), Alpers Syndrome and Kuru, the normal cellular form of this protein (PrPc) is transformed into an altered protein when it comes into contact with an infectious prion protein (PrPsc) from another host. This altered PrPsc accumulates in cytoplasmic vesicles of diseased individuals forming lesions, vacuoles and amyloid deposits. View All» Epitope: N-terminus, a.a. 78-97 View All» Immunogen: Synthetic peptide that corresponds to amino acids 79-97 of the N-terminus of the human PrP27-30. View All» Species Reactivity: Human View All» Application Notes: Immunohistochemistry: >1:200 on paraffin embedded, formalin fixed human brain. Western blots: > 1:2,000 ELISA: > 1:35,000 Optimal working dilutions must be determined by the end user. View All» Presentation: Goat serum. Liquid containing 0.01% thimerosal View All» Storage Conditions: Maintain at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. View All» UniProt Number: P04156 View All» Entrez Gene Number:

• NM_000311.3

• NM_001080121.1

• NM_001080122.1

• NM_001080123.1

• NM_183079.2
  View All» Gene Symbol:
  • PRNP

  • CD230

  • PrP33-35C

  • PRP

  • ASCR

  • PrP27-30

  • GSS

  • CJD

  • PRIP

  • PrP

  • PrPc

  • MGC26679
    View All» Alternate Names: PrP; CD230 View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
    • ELISA

    • Western Blotting

    • Immunohistochemistry (Paraffin)
      View All» Entrez Gene Summary: 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. Alternative splicing results in multiple transcript variants encoding the same protein. View All» UniProt Summary: FUNCTION: SwissProt: P04156 # The physiological function of PrP is not known.SIZE: 253 amino acids; 27661 Da SUBUNIT: PrP has a tendency to aggregate yielding polymers called rods.SUBCELLULAR LOCATION: Cell membrane; Lipid-anchor, GPI-anchor.PTM: The glycosylation pattern (the amount of mono-, di- and non- glycosylated forms or glycoforms) seems to differ in normal and CJD prion.DISEASE: SwissProt: P04156 # PrP is found in high quantity in the brain of humans and animals infected with neurodegenerative diseases known as transmissible spongiform encephalopathies or prion diseases, like: Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann-Straussler disease (GSD), Huntington disease-like 1 (HDL1) and kuru in humans; scrapie in sheep and goat; bovine spongiform encephalopathy (BSE) in cattle; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of mule deer and elk; feline spongiform encephalopathy (FSE) in cats and exotic ungulate encephalopathy (EUE) in nyala and greater kudu. The prion diseases illustrate three manifestations of CNS degeneration: (1) infectious (2) sporadic and (3) dominantly inherited forms. TME, CWD, BSE, FSE, EUE are all thought to occur after consumption of prion-infected foodstuffs. & Defects in PRNP are the cause of Creutzfeldt-Jakob disease (CJD) [MIM:123400]. CJD 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 annimal 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. & Defects in PRNP are the cause of fatal familial insomnia (FFI) [MIM:600072]. FFI is an autosomal dominant disorder and is characterized by neuronal degeneration limited to selected thalamic nuclei and progressive insomnia. & Defects in PRNP are the cause of Gerstmann-Straussler disease (GSD) [MIM:137440]. GSD is a heterogeneous disorder and was defined as a spinocerebellar ataxia with dementia and plaquelike deposits. GSD incidence is less than 2 per 100 million live births. & Defects in PRNP are the cause of Huntington disease-like 1 (HDL1) [MIM:603218]. HDL1 is an autosomal dominant, early onset neurodegenerative disorder with prominent psychiatric features. & Defects in PRNP are the cause of kuru [MIM:245300]. 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. & Defects in PRNP are the cause of prion disease with protracted course [MIM:606688]; an 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.SIMILARITY:SwissProt: P04156 ## Belongs to the prion family. View All» Brand Family: Chemicon View All» Product Name: Anti-Prion Protein Antibody, N-terminus, a.a. 78-97 | AB5058 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 200 µL View All» Format: Serum View All» Host: Goat View All»
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