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.
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Linkage
The action of this antibody can be blocked using blocking peptide SBP3500449.
Physical form
Supplied in PBS with 0.02% sodium azide.
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.
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.
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
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
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