SAB4200388

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

~1.5 mg/mL, affinity isolated antibody

• 동의어(들): Anti-Dysferlin (N-terminal region) antibody produced in rabbit, Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
• UNSPSC 코드: 12352203
• NACRES: NA.41

속성

• 생물학적 소스: rabbit
• Quality Level: 200
• 결합: unconjugated
• 항체 형태: affinity isolated antibody
• 항체 생산 유형: primary antibodies
• 클론: polyclonal
• 양식: buffered aqueous solution
• 분자량: ~250 kDa
• 종 반응성: human, mouse
• 향상된 검증: recombinant expression; Learn more about Antibody Enhanced Validation
• 농도: ~1.5 mg/mL
• 기술: immunocytochemistry: 7-14 μg/mL using differentiated C2C12 myoblasts; immunoprecipitation (IP): 5-10 μg using HEK-293T cells over-expressing human dysferlin; western blot: 0.5-1.0 μg/mL using HEK-293T cells over-expressing human dysferlin
• 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)

설명

일반 설명

Dysferlin is a transmembrane protein, that belongs to the ferlin-1 family of proteins including myoferlin and otoferlin. It is homologous to the C. elegans fer-1 protein. Dysferlin is expressed in kidney cells, skeletal and cardiac muscle.

Dysferlin is encoded by the gene mapped to human chromosome 2p13.2

특이성

Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.

면역원

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.

애플리케이션

Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:

• immunoblotting
• immunoprecipitation
• immunofluorescence

생화학적/생리학적 작용

Dysferlin is implicated in membrane fusion events. It plays a role in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and -3. Mutations in the dysferlin gene are associated with limb-girdle muscle dystrophy type 2B (LGMD2B), distal anterior compartment myopathy and related Miyoshi myopathy.

물리적 형태

solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

저장 및 안정성

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.

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