추천 제품
생물학적 소스
rabbit
결합
unconjugated
항체 형태
affinity isolated antibody
항체 생산 유형
primary antibodies
클론
polyclonal
형태
buffered aqueous solution
분자량
antigen 237 kDa
종 반응성
mouse, human
농도
~1 mg/mL
기술
ELISA: 1:10000
western blot: 1:500-1:1000
NCBI 수납 번호
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... DYSF(8291)
일반 설명
Dysferlin (DYSF) is a large transmembrane protein that belongs to the ferlin family of Ca2+-dependent phospholipid-binding proteins. It is expressed in skeletal and cardiac muscles. DYSF gene is mapped to human chromosome 2p13. Anti-Dysferlin antibody detects endogenous levels of total dysferlin protein.
면역원
The antiserum was produced against synthesized peptide derived from human Dysferlin.
Immunogen Range: 1981-2030
Immunogen Range: 1981-2030
애플리케이션
Anti-Dysferlin, antibody produced in rabbit has been used in western blotting.
생화학적/생리학적 작용
Dysferlin (DYSF) plays a key role in membrane repair, vesicle trafficking, and Ttubule structure. Lack of DYSF protein expression in skeletal muscles leads to dysferlinopathy. Mutations in the DYSF gene are associated with Miyoshi myopathy, limb-girdle muscular dystrophy type 2B (LGMD2B), and distal myopathy with anterior tibial onset (DMAT).
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.
물리적 형태
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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
nwg
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
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.
Jin Tang et al.
Neuropathology : official journal of the Japanese Society of Neuropathology (2018-05-26)
Dysferlinopathy, a progressive muscular dystrophy, results from mutations in the Dysferlin gene (DYSF, MIM*603009). Traditional diagnosis relies on the reduction or absence of dysferlin. However, altered dysferlin has been observed in other myopathies, leading to a precise diagnosis through molecular
Functional recovery of a novel knock-in mouse model of dysferlinopathy by readthrough of nonsense mutation
Seo K, et al.
Molecular therapy. Methods & clinical development (2021)
Kyowon Seo et al.
Molecular therapy. Methods & clinical development, 21, 702-709 (2021-06-19)
Biallelic mutations in the dysferlin gene cause limb-girdle muscular dystrophy 2B or Miyoshi distal myopathy. We found that nonsense mutations are the most common mutation type among Korean patients with dysferlinopathy; more than half of the patients have at least
Janice A Dominov et al.
Annals of clinical and translational neurology, 6(4), 642-654 (2019-04-26)
Dysferlin is a large transmembrane protein that functions in critical processes of membrane repair and vesicle fusion. Dysferlin-deficiency due to mutations in the dysferlin gene leads to muscular dystrophy (Miyoshi myopathy (MM), limb girdle muscular dystrophy type 2B (LGMD2B), distal
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