生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
形式
buffered aqueous solution
分子量
predicted mol wt 53 kDa
种属反应性
mouse, rat, human
技术
immunofluorescence: suitable
immunohistochemistry: suitable
indirect ELISA: suitable
western blot: suitable
NCBI登记号
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... SCARB2(950)
一般描述
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.
免疫原
LIMP2 antibody was raised against a 16 amino acid peptide from near the center of human LIMP2.
应用
Anti-LIMP2 antibody produced in rabbit has been used in immunofluorescence (1:100) and western blotting.
生化/生理作用
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.
特点和优势
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.
联系
The action of this antibody can be blocked using blocking peptide SBP3500449.
外形
Supplied in PBS with 0.02% sodium azide.
免责声明
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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相关产品
WGK
WGK 2
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
常规特殊物品
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
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)
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