Dysferlin (DYSF) Mouse Monoclonal Antibody [Clone ID: Ham3/17B2]

CAT#: TA355285

Dysferlin Mouse Monoclonal Antibody



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CNY 20,141.00


货期*
7周

规格
    • 1 ml

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Specifications

Product Data
Clone Name Ham3/17B2
Applications IHC
Recommend Dilution 1:20 - 1:40
Reactivity Human
Host Mouse
Clonality Monoclonal
Immunogen Synthetic peptide containing amino acids 349-366, spanning exons 11 and 12, of the human dysferlin molecule
Specificity Reactive with the dysferlin molecule in human skeletal muscle. Also present in many non-muscle tissues
Formulation Lyophilized tissue culture supernatant containing sodium azide as a preservative.
Reconstitution Method The user is required to reconstitute the contents of the vial with the correct volume of sterile distilled water as indicated on the vial label
Conjugation Unconjugated
Storage Condition Store at 2-8°C
Gene Name dysferlin
Background Dysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. In patients with LGMD2B and MM, immunoreactivity to dysferlin is severely reduced or lost. Patients with other neuromuscular conditions demonstrate normal labeling patterns.
Synonyms dysferlin; FER1L1; FLJ00175; FLJ90168; LGMD2B; OTTHUMP00000202233; OTTHUMP00000202234; OTTHUMP00000202235; OTTHUMP00000202236; OTTHUMP00000202237; OTTHUMP00000202240
Reference Data
*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.
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