SAB4200453

Anti-Dysferlin (N-terminal) antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody, antigen mol wt ~250 kDa

• 동의어(들): Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
• UNSPSC 코드: 12352203
• NACRES: NA.41

속성

• 생물학적 소스: rabbit
• 결합: unconjugated
• 항체 형태: affinity isolated antibody
• 항체 생산 유형: primary antibodies
• 클론: polyclonal
• 형태: buffered aqueous solution
• 분자량: antigen ~250 kDa
• 종 반응성: rat, human, mouse
• 향상된 검증: recombinant expression; Learn more about Antibody Enhanced Validation
• 농도: ~1.0 mg/mL
• 기술: dot blot: 0.1-0.2 μg/mL using extracts of HEK-293T cells over-expressing human dysferlin and 1-2 mg/mL using A10 cells.; immunohistochemistry: 20-30 μg/mL using methanol-acetone fixed frozen sections of mouse skeletal muscle.; indirect immunofluorescence: 5-10 μg/mL using differentiated C2C12 myoblasts.
• UniProt 수납 번호: O75923
• 배송 상태: dry ice
• 저장 온도: −20°C
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... DYSF(8291) ; mouse ... Dysf(26903) ; rat ... Dysf(312492)

설명

일반 설명

Dysferlin is a 230 kDa transmembrane protein that belongs to the ferlin family of proteins including myoferlin and otoferlin and is homologous to the C. elegans fer-1 protein. It is expressed early during human development.

면역원

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin, conjugated to KLH. The corresponding sequence is identical in human dysferlin isoforms 1-14 and identical in mouse dysferlin.

애플리케이션

Anti-Dysferlin (N-terminal) antibody produced in rabbit has been used in several immunochemical techniques including immunoblotting, immunofluorescence and immunohistochemistry.

생화학적/생리학적 작용

Dysferlin is implicated in membrane fusion events. It has also been involved in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane protein caveolin 3 have been shown to regulate the endocytosis of dysferlin. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and 3, resulting in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex. Mutations in the dysferlin gene leads to the development of limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy.

물리적 형태

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM 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.

안전성 정보

• Storage Class Code: 10 - Combustible liquids
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable