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Merck
모든 사진(4)

주요 문서

HPA004128

Sigma-Aldrich

Anti-PLP1 antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-GPM6C, Anti-PLP, Anti-SPG2

로그인조직 및 계약 가격 보기


About This Item

UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
NACRES:
NA.41

생물학적 소스

rabbit

Quality Level

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

양식

buffered aqueous glycerol solution

종 반응성

human

향상된 검증

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

기술

immunohistochemistry: 1:500-1:1000

면역원 서열

LLLAEGFYTTGAVRQIFGDYKTTICGKGLSATVTGGQKGRGSRGQHQAHSLERVCHCLGKWLGHPDKFVGI

UniProt 수납 번호

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... PLP1(5354)

일반 설명

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.

면역원

Myelin proteolipid protein recombinant protein epitope signature tag (PrEST)

생화학적/생리학적 작용

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.

특징 및 장점

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:
  • 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.

결합

Corresponding Antigen APREST74534

물리적 형태

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

법적 정보

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

면책조항

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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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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시험 성적서(COA)

Lot/Batch Number

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문서 라이브러리에서 최근에 구매한 제품에 대한 문서를 찾아보세요.

문서 라이브러리 방문

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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