HPA004128
Anti-PLP1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Synonym(s):
Anti-GPM6C, Anti-PLP, Anti-SPG2
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About This Item
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biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
enhanced validation
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
technique(s)
immunohistochemistry: 1:500-1:1000
immunogen sequence
LLLAEGFYTTGAVRQIFGDYKTTICGKGLSATVTGGQKGRGSRGQHQAHSLERVCHCLGKWLGHPDKFVGI
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... PLP1(5354)
General description
Proteolipid protein 1 (PLP1) gene encodes for two major proteins of the central nervous system (CNS) myelin, such as proteolipid protein (PLP) and DM20. This gene is located on the human chromosome at Xq22.2.
Immunogen
Myelin proteolipid protein recombinant protein epitope signature tag (PrEST)
Biochem/physiol Actions
Proteolipid protein (PLP) is involved in membrane adhesion, compaction of myelin and synthesis of myelin intra-period line. It is also involved in the maturation of oligodendrocytes. PLP plays a role in wrapping, maintenance and survival of axons. Mutations in the proteolipid protein 1 (PLP1) gene is associated with Pelizaeus-Merzbacher disease indicated by nystagmus, spasticity, microcephaly, ataxia and intellectual disability. PLP1 gene plays a role in Xq22.2 microdeletion and microduplication syndromes. Duplication of this gene results in the overexpression of PLP, which leads to accumulation in the cytoplasm myelinating oligodendrocytes and defects in myelin.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Linkage
Corresponding Antigen APREST74534
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
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.
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wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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Find documentation for the products that you have recently purchased in the Document Library.
Ravinder Pannu et al.
Journal of neuroscience research, 79(3), 340-350 (2004-12-18)
Spinal cord injury (SCI) is a devastating and complex clinical condition involving proinflammatory cytokines and nitric oxide toxicity that produces a predictable pattern of progressive injury entailing neuronal loss, axonal destruction, and demyelination at the site of impact. The involvement
Derek P Ng et al.
Biochemistry, 53(23), 3747-3757 (2014-05-27)
Central to the formation of tertiary structure in membrane protein folding is the presence of amino acid sequence motifs (such as "small-XXX-small" segments) in the TM segments that promote interaction-compatible surfaces through which the TM α-helices interact. Here, we sought
P Martínez-Montero et al.
Clinical genetics, 84(6), 566-571 (2013-01-26)
Pelizaeus-Merzbacher disease (PMD) is caused in most cases by either duplications or point mutations in the PLP1 gene. This disease, a dysmyelinating disorder affecting mainly the central nervous system, has a wide clinical spectrum and its causing mutations act through
Dorota Hoffman-Zacharska et al.
Medycyna wieku rozwojowego, 17(4), 293-300 (2014-02-13)
The Pelizaeus-Merzbacher disease (PMD) is a rare X-linked recessive hypomyelination disorder caused by mutations of the proteolipid protein1 gene (PLP1). There is a spectrum of PLP1-related disorders from very severe connatal PMD, through classical PMD to mild spastic paraplegia type
A microdeletion at Xq22. 2 implicates a glycine receptor GLRA4 involved in intellectual disability, behavioral problems and craniofacial anomalies
Labonne J D J, et al.
BMC Neurology, 16(1), 1-12 (2016)
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