SAB3500449
Anti-LIMP2 antibody produced in rabbit
affinity isolated antibody, buffered aqueous solution
동의어(들):
Anti-CD36L2, Anti-Lysosomal integral membrane protein 2, Anti-SCARB2, Anti-SR-B2, Anti-Scavenger receptor class B member 2
로그인조직 및 계약 가격 보기
크기 선택
100 μG
₩831,184
₩831,184
구입 가능 여부는 고객센터에 문의하십시오.
모든 사진(3)
크기 선택
보기 변경
100 μG
₩831,184
About This Item
UNSPSC 코드:
12352203
NACRES:
NA.41
₩831,184
구입 가능 여부는 고객센터에 문의하십시오.
추천 제품
생물학적 소스
rabbit
Quality Level
결합
unconjugated
항체 형태
affinity isolated antibody
항체 생산 유형
primary antibodies
클론
polyclonal
양식
buffered aqueous solution
분자량
predicted mol wt 53 kDa
종 반응성
mouse, rat, human
기술
immunofluorescence: suitable
immunohistochemistry: suitable
indirect ELISA: suitable
western blot: suitable
NCBI 수납 번호
UniProt 수납 번호
배송 상태
dry ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... SCARB2(950)
일반 설명
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.
면역원
LIMP2 antibody was raised against a 16 amino acid peptide from near the center of human LIMP2.
애플리케이션
Anti-LIMP2 antibody produced in rabbit has been used in immunofluorescence (1:100) and western blotting.[1]
생화학적/생리학적 작용
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.
특징 및 장점
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.
결합
The action of this antibody can be blocked using blocking peptide SBP3500449.
물리적 형태
Supplied in PBS with 0.02% sodium azide.
면책조항
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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관련 제품
Storage Class Code
10 - Combustible liquids
WGK
WGK 2
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
가장 최신 버전 중 하나를 선택하세요:
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
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
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)
활성 필터
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