Rabbit LIMP2 Polyclonal Antibody | anti-SCARB2 antibody

LIMP2 Antibody

Cat. Num. AAA150945

• Gene Names: SCARB2; AMRF; EPM4; LGP85; CD36L2; HLGP85; LIMP-2; LIMPII; SR-BII
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: LIMP2 Antibody is affinity chromatography purified via peptide column.
• Synonyms: LIMP2; Polyclonal Antibody; LIMP2 Antibody; AMRF; EPM4; LGP85; CD36L2; HLGP85; LIMP-2; LIMPII; SR-BII; Lysosome membrane protein 2; 85 kDa lysosomal membrane sialoglycoprotein; scavenger receptor class B; member 2; anti-SCARB2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: LIMP2 Antibody is affinity chromatography purified via peptide column.
• Form/Format: Liquid
• Concentration: 1 mg/mL (varies by lot)
• Sequence Length: 478

• Applicable Applications for anti-SCARB2 antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: LIMP2 antibody can be used for detection of LIMP2 by Western blot at 1 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 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.
• Conjugate: Unconjugated
• Immunogen: LIMP2 antibody was raised against a 16 amino acid synthetic peptide from near the center of human LIMP2.
• Buffer: LIMP2 Antibody is supplied in PBS containing 0.02% sodium azide.
• Preparation and Storage: 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.

Western Blot (WB)

(Western blot analysis of LIMP2 in mouse liver tissue lysate with LIMP2 antibody at 1 μg/mL in (A) the absence and (B) presence of blocking peptide.)

Immunohistochemistry (IHC)

(Immunohistochemistry of LIMP2 in human skeletal muscle tissue with LIMP2 antibody at 2.5 μg/mL.)

Immunofluorescence (IF)

(Immunofluorescence of LIMP2 in Human Skeletal Muscle tissue with LIMP2 antibody at 20 μg/mL.)

Related Product Information for anti-SCARB2 antibody

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 and Uniprot Product Information

• NCBI GI #: 18257312
• NCBI GeneID: 950
• UniProt Accession #: Q14108; Q53Y63; B4DKD8; E7EM68
• Molecular Weight: 37,767 Da
• NCBI Official Full Name: Scavenger receptor class B, member 2
• NCBI Official Synonym Full Names: scavenger receptor class B, member 2
• NCBI Official Symbol: SCARB2
• NCBI Official Synonym Symbols: AMRF; EPM4; LGP85; CD36L2; HLGP85; LIMP-2; LIMPII; SR-BII
• NCBI Protein Information: 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)
• UniProt Protein Name: Lysosome membrane protein 2
• Protein Family: Lysosome membrane protein
• UniProt Gene Name: SCARB2
• UniProt Synonym Gene Names: CD36L2; LIMP2; LIMPII; LGP85; LIMP II
• UniProt Entry Name: SCRB2_HUMAN

NCBI Description

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 Description

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; Membrane protein, integral; Receptor, misc.

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