Key Documents

Anti-LIMP2 antibody produced in rabbit

Name: Anti-LIMP2 antibody produced in rabbit
Brand: SIGMA
Price: 584 USD

affinity isolated antibody, buffered aqueous solution

Synonym(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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Pack Size	SKU	Availability	Price
100 μg		Usually ships in 5 business days. (Orders outside of US, please allow an additional 1-2 weeks for delivery)	$584.00

About This Item

NACRES:

NA.41

UNSPSC Code:

12352203

Conjugate:

unconjugated

Clone:

polyclonal

Application:

ELISA (i), IF, IHC, WB

Citations:

$584.00

Usually ships in 5 business days. (Orders outside of US, please allow an additional 1-2 weeks for delivery)

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biological source

rabbit

Quality Level

100

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

predicted mol wt 53 kDa

species reactivity

mouse, rat, human

technique(s)

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

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Primary Antibodies

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Show Differences

1 of 1

This Item	ABC822	L0419	SAB2501242
Sigma-Aldrich SAB3500449 Anti-LIMP2 antibody produced in rabbit	Sigma-Aldrich ABC822 Anti-LIMP II Antibody	Sigma-Aldrich L0419 Anti-LAMP1−Cy3^™ antibody produced in rabbit	Sigma-Aldrich SAB2501242 Anti-SCARB2 antibody produced in goat
biological source rabbit	biological source rabbit	biological source rabbit	biological source goat
conjugate unconjugated	conjugate -	conjugate CY3 conjugate	conjugate unconjugated
Quality Level 100	Quality Level 100	Quality Level 200	Quality Level 100
antibody form affinity isolated antibody	antibody form affinity isolated antibody	antibody form affinity isolated antibody	antibody form affinity isolated antibody
species reactivity mouse, rat, human	species reactivity human	species reactivity mouse, human, rat	species reactivity mouse, human
form buffered aqueous solution	form -	form buffered aqueous solution	form buffered aqueous solution

General description

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.

Immunogen

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

Application

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

Biochem/physiol Actions

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.

Features and Benefits

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.

Physical form

Supplied in PBS with 0.02% sodium azide.

Other Notes

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

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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Product No.

Description

Pricing

SBP3500449

LIMP2 Blocking Peptide for SAB3500449

Storage Class

10 - Combustible liquids

wgk

WGK 2

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number

46210802

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Disease-causing mutations within the lysosomal integral membrane protein type 2 (LIMP-2) reveal the nature of binding to its ligand beta-glucocerebrosidase.

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

Progressive myoclonus epilepsy without renal failure in a Chinese family with a novel mutation in SCARB2 gene and literature review.

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

LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase.

Reczek, et al.

Cell, 131, 770-783 (2017)

A novel homozygous splice-site mutation in SCARB2 is associated with progressive myoclonic epilepsy with renal failure.

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

Global Trade Item Number

SKU	GTIN
SBP3500449-0.05MG	04061832169453
SAB3500449-100UG	04061832496214

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