LIMP2 Antibody | MyBioSource MBS150433 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

LIMP2 Antibody

catalog :

MBS150433

quantity :

0.1 mg

price :

345 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS150433

products type :

Antibody

products full name :

LIMP2 Antibody

products short name :

LIMP2

products name syn :

LIMP2; AMRF; EPM4; LGP85; CD36L2; HLGP85; LIMP-2; LIMPII; SR-BII; LIMP2; Lysosome membrane protein 2; 85 kDa lysosomal membrane sialoglycoprotein; scavenger receptor class B, member 2

other names :

Scavenger receptor class B, member 2; Lysosome membrane protein 2; lysosome membrane protein 2; LIMP II; CD36 antigen-like 2; lysosome membrane protein II; 85 kDa lysosomal membrane sialoglycoprotein; 85 kDa lysosomal sialoglycoprotein scavenger receptor class B, member 2; CD36 antigen (collagen type I receptor, thrombospondin receptor)-like 2 (lysosomal integral membrane protein II); scavenger receptor class B, member 2; 85 kDa lysosomal membrane sialoglycoprotein; LGP85; CD36 antigen-like 2; Lysosome membrane protein II; LIMP II; Scavenger receptor class B member 2; CD_antigen: CD36

products gene name :

SCARB2

other gene names :

SCARB2; SCARB2; AMRF; EPM4; LGP85; CD36L2; HLGP85; LIMP-2; LIMPII; SR-BII; CD36L2; LIMP2; LIMPII; LGP85; LIMP II

uniprot entry name :

SCRB2_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse

sequence length :

478

purity :

LIMP2 Antibody is affinity chromatography purified via peptide column.

form :

Liquid

concentration :

1 mg/mL

storage stability :

LIMP2 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

tested application :

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

app notes :

LIMP2 antibody can be used for detection of LIMP2 by Western blot at 1 and 2 mug/mL. Despite its predicted molecular weight, LIMP2 runs at approximately 80 - 85 kDa in SDS-PAGE. Antibody can also be used for immunohistochemistry starting at 10 mug/mL.

other info1 :

Conjugate: Unconjugated. Immunogen: LIMP2 antibody was raised against a 18 amino acid synthetic peptide from near the carboxy terminus of human LIMP2. Buffer: LIMP2 Antibody is supplied in PBS containing 0.02% sodium azide.

products description :

LIMP2 Antibody: The lysosomal integral membrane protein 2 (LIMP2) is a heavily glycosylated type III transmembrane protein, the majority of which exists in the lumen of the lysosome and a cytoplasmic domain of approximately 20 amino acids. A deficiency of LIMP2 in mice causes uretic pelvic junction obstruction, deafness, and peripheral neuropathy associated with impaired vesicular trafficking and distribution of apically expressed proteins. More recently, LIMP2 was shown to act as a receptor to bind beta-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted beta-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor.

ncbi gi num :

18257312

ncbi acc num :

AAH21892

uniprot acc num :

Q14108

ncbi mol weight :

37,767 Da

ncbi pathways :

Lysosome Pathway (99052); Lysosome Pathway (96865)

ncbi summary :

The protein encoded by this gene is a type III glycoprotein that is located primarily in limiting membranes of lysosomes and endosomes. Earlier studies in mice and rat suggested that this protein may participate in membrane transportation and the reorganization of endosomal/lysosomal compartment. The protein deficiency in mice was reported to impair cell membrane transport processes and cause pelvic junction obstruction, deafness, and peripheral neuropathy. Further studies in human showed that this protein is a ubiquitously expressed protein and that it is involved in the pathogenesis of HFMD (hand, foot, and mouth disease) caused by enterovirus-71 and possibly by coxsackievirus A16. Mutations in this gene caused an autosomal recessive progressive myoclonic epilepsy-4 (EPM4), also known as action myoclonus-renal failure syndrome (AMRF). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Feb 2011]

uniprot summary :

SCARB2: Acts as a lysosomal receptor for glucosylceramidase (GBA) targeting. Defects in SCARB2 are the cause of progressive myoclonic epilepsy type 4 with or without renal failure (EPM4). An autosomal recessive progressive myoclonic epilepsy associated with renal failure in some cases. Cognitive function is preserved. Myoclonus is a brief, involuntary twitching of a muscle or a group of muscles. Cognitive function is preserved. Genetic variants in SCARB2 can act as modifier of the phenotypic expression and severity of Gaucher disease. Belongs to the CD36 family. Protein type: Membrane protein, multi-pass; Receptor, misc.; Membrane protein, integral. Chromosomal Location of Human Ortholog: 4q21.1. Cellular Component: lysosomal lumen; focal adhesion; membrane; lysosomal membrane; integral to membrane. Molecular Function: protein binding; enzyme binding; receptor activity. Biological Process: protein targeting to lysosome; cell adhesion. Disease: Epilepsy, Progressive Myoclonic 4, With Or Without Renal Failure

size1 :

0.1 mg

price1 :

345 USD

more info or order :

MyBioSource product webpage