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SAB3500449

Sigma-Aldrich

Anti-LIMP2 antibody produced in rabbit

affinity isolated antibody, buffered aqueous solution

Sinônimo(s):

Anti-CD36L2, Anti-Lysosomal integral membrane protein 2, Anti-SCARB2, Anti-SR-B2, Anti-Scavenger receptor class B member 2

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100 μG
R$ 4.122,00

R$ 4.122,00

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100 μG
R$ 4.122,00

About This Item

Código UNSPSC:
12352203
NACRES:
NA.41

R$ 4.122,00

Check Cart for Availability

fonte biológica

rabbit

Nível de qualidade

100

conjugado

unconjugated

forma do anticorpo

affinity isolated antibody

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

Formulário

buffered aqueous solution

peso molecular

predicted mol wt 53 kDa

reatividade de espécies

mouse, rat, human

técnica(s)

immunofluorescence: suitable
immunohistochemistry: suitable
indirect ELISA: suitable
western blot: suitable

nº de adesão NCBI

AAH21892

nº de adesão UniProt

Q14108

Condições de expedição

dry ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... SCARB2(950)

Descrição geral

Lysosomal integral membrane protein type 2 (LIMP2) is an N-glycosylated type III transmembrane protein. It is composed of a lumenal domain, two transmembrane domains, and a cytoplasmic domain. LIMP2 belongs to the CD36 family of scavenger receptor proteins and is found abundantly in the lysosome. The LIMP2 gene is located on the human chromosome at 4q21.1.

Imunogênio

LIMP2 antibody was raised against a 16 amino acid peptide from near the center of human LIMP2.

Aplicação

Anti-LIMP2 antibody produced in rabbit has been used in immunofluorescence (1:100) and western blotting.[1]

Ações bioquímicas/fisiológicas

Lysosomal integral membrane protein type 2 (LIMP2) binds to β-glucocerebrosidase. Mutations in the LIMP 2 gene are associated with action myoclonus-renal failure syndrome, a fatal autosomal recessive disorder. Higher levels of LIMP2 lead to enlargement of early or late endosomes/lysosomes. Overexpression of LIMP2 is associated with impairment of endocytotic membrane trafficking.

Características e benefícios

Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.

Ligação

The action of this antibody can be blocked using blocking peptide SBP3500449.

forma física

Supplied in PBS with 0.02% sodium azide.

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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Preços

Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 2

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable

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Certificados de análise (COA)

Lot/Batch Number
46210802

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Judith Blanz et al.
Human molecular genetics, 19(4), 563-572 (2009-11-26)
Action myoclonus-renal failure syndrome (AMRF) is caused by mutations in the lysosomal integral membrane protein type 2 (LIMP-2/SCARB2). LIMP-2 was identified as a sorting receptor for beta-glucocerebrosidase (beta-GC), which is defective in Gaucher disease. To date, six AMRF-causing mutations have
Abolfazl Yari et al.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (2021-03-28)
Progressive myoclonic epilepsy-4 with or without renal failure (EPM4) is a rare neurological autosomal recessive disorder caused by mutations in SCARB2 gene. In this study, we described clinical features and genetic causes of an Iranian family with two affected individuals
LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase.
Reczek, et al.
Cell, 131, 770-783 (2017)
Wo-Tu Tian et al.
Seizure, 57, 80-86 (2018-04-02)
To describe the clinical and genetic features of a Chinese progressive myoclonus epilepsy (PME) patient related with SCARB2 mutation without renal impairment and review 27 SCARB2-related PME patients from 11 countries. The patient was a 27-year-old man with progressive action

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