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Merck
모든 사진(5)

Key Documents

HPA011422

Sigma-Aldrich

Anti-SGCB antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-43 kDa dystrophin-associated glycoprotein, Anti-43DAG, Anti-A3b, Anti-Beta-SG, Anti-Beta-sarcoglycan

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모든 사진(5)

About This Item

UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
HPA011422
NACRES:
NA.41

생물학적 소스

rabbit

Quality Level

100

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

형태

buffered aqueous glycerol solution

종 반응성

human

향상된 검증

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

기술

immunohistochemistry: 1:200- 1:500

면역원 서열

FHMGGNMELKAENSIILNGSVMVSTTRLPSSSSGDQLGSGDWVRYKLCMCADGTLFKVQVTSQNMGCQISDNPCGNTH

UniProt 수납 번호

Q16585

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... SGCB(6443)

관련 카테고리

면역원

Beta-sarcoglycan recombinant protein epitope signature tag (PrEST)

애플리케이션

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

생화학적/생리학적 작용

SGCB (Sarcoglycan, β) is a 43kD glycosylated transmembrane, dystrophin-associated glycoprotein. It is ubiquitously expressed, but mainly in skeletal and cardiac muscle. It forms and maintains the assembly and functional stability of the sarcoglycan complex by forming disulfide-bond. The complex includes α, β, γ, and δ, which are tightly bound to each other. The mutation in the sarcoglycan complex is associated with four types of autosomal recessive limb-girdle muscular dystrophies (LGMD2C-F).

특징 및 장점

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

결합

Corresponding Antigen APREST72131

물리적 형태

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

법적 정보

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

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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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

시험 성적서(COA)

제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.

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문서 라이브러리 방문

F Duclos et al.
Neuromuscular disorders : NMD, 8(1), 30-38 (1998-05-05)
The sarcoglycan complex is involved in the etiology of four autosomal recessive limb-girdle muscular dystrophies (LGMD2C-F). A missense mutation (T151R) in the beta-sarcoglycan gene on chromosome 4q12 has been shown to cause a mild form of LGMD2E in 11 families
C G Bönnemann et al.
Neuromuscular disorders : NMD, 8(3-4), 193-197 (1998-06-19)
Four of the currently recognized autosomal recessive limb-girdle muscular dystrophies (LGMD type 2C-F) are caused by mutations in the genes encoding components of the sarcoglycan complex. LGMD 2C, caused by mutations in gamma-sarcoglycan, is prevalent in northern Africa, especially in
R Barresi et al.
Journal of medical genetics, 37(2), 102-107 (2000-02-09)
Two young males with limb-girdle muscular dystrophy (LGMD) resulting from sarcoglycan deficiency died at 27 (patient 1) and 18 years (patient 2) of severe cardiomyopathy. Genetic analysis showed that they were compound heterozygotes for mutations in the beta sarcoglycan gene.
Sara F Henriques et al.
PloS one, 13(1), e0191274-e0191274 (2018-01-24)
Sarcoglycanopathies are rare autosomic limb girdle muscular dystrophies caused by mutations in one of the genes coding for sarcoglycan (α, β, δ, and γ-sarcoglycans). Sarcoglycans form a complex, which is an important part of the dystrophin-associated glycoprotein complex that protects

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