Dysferlin (DYSF) Mouse Monoclonal Antibody [Clone ID: Ham1/7B6]

Specifications

Product Data
Clone Name	Ham1/7B6
Applications	IHC
Recommend Dilution	1:20 - 1:40
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Synthetic peptide containing amino acids 1999-2016 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
Database Link	Entrez Gene 8291 Human UniProt ID O75923
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

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