HPA004128
Anti-PLP1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinônimo(s):
Anti-GPM6C, Anti-PLP, Anti-SPG2
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About This Item
Produtos recomendados
fonte biológica
rabbit
Nível de qualidade
conjugado
unconjugated
forma do anticorpo
affinity isolated antibody
tipo de produto de anticorpo
primary antibodies
clone
polyclonal
linha de produto
Prestige Antibodies® Powered by Atlas Antibodies
Formulário
buffered aqueous glycerol solution
reatividade de espécies
human
validação aprimorada
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
técnica(s)
immunohistochemistry: 1:500-1:1000
sequência de imunogênio
LLLAEGFYTTGAVRQIFGDYKTTICGKGLSATVTGGQKGRGSRGQHQAHSLERVCHCLGKWLGHPDKFVGI
nº de adesão UniProt
Condições de expedição
wet ice
temperatura de armazenamento
−20°C
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... PLP1(5354)
Descrição geral
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.
Imunogênio
Myelin proteolipid protein recombinant protein epitope signature tag (PrEST)
Ações bioquímicas/fisiológicas
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.
Características e benefícios
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.
Ligação
Corresponding Antigen APREST74534
forma física
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Informações legais
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Exoneração de responsabilidade
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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Código de classe de armazenamento
10 - Combustible liquids
Classe de risco de água (WGK)
WGK 1
Ponto de fulgor (°F)
Not applicable
Ponto de fulgor (°C)
Not applicable
Equipamento de proteção individual
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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Certificados de análise (COA)
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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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