Emerin (EMD) Mouse Monoclonal Antibody [Clone ID: 4G5]
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Specifications
Product Data | |
Clone Name | 4G5 |
Applications | IHC |
Recommended Dilution | 1:160 |
Reactivities | Human |
Host | Mouse |
Isotype | IgG1 |
Clonality | Monoclonal |
Immunogen | Prokaryotic recombinant protein corresponding to a 222 amino acid region near the N-terminus of the emerin protein |
Specificity | Human emerin protein |
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 | Store at 2-8°C |
Stability | 12 months |
Gene Name | emerin |
Database Link | |
Background | Emery-Dreifuss muscular dystrophy (EDMD) is a late onset, X-linked, recessive disorder characterized by slowly progressing contractures, wasting of skeletal muscle and cardiomyopathy usually presented as heart block. Contractures are seen in the elbows, Achilles tendons and post cervical muscles with humero-peroneal distribution early in the course of the disease. The STA gene, at Xq28 locus, encodes a serine-rich 34kD protein, emerin, which is ubiquitous in tissues and is found in highest concentration in skeletal and cardiac muscle. Emerin is localized in the nuclear membrane of normal muscle cells and its deficiency plays a crucial part in the pathology of EDMD. |
Synonyms | EDMD; emerin; LEMD5; STA |
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