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Emerin (EDMD, EMD, LEM Domain Containing 5, LEMD5, STA)

Emerin (EDMD, EMD, LEM Domain Containing 5, LEMD5, STA)

Cat no: E2257-01E

Supplier: United States Biological
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Emerin is the product of the STA gene and is found ubiquitously in tissues. Emerin is found in the highest concentrations in skeletal and cardiac muscle and d e f iciency in its expression is a key factor in Emery- Dreifuss muscular dystrophy. Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1:250-1:500. Immunohistochemistry (Frozen): 1:20-1:40 for 1 hr. at RT Optimal dilutions to be determined by the researcher. Positive Control: Western Blot: Sup T cells Immunohistochemistry: Present in most tissues (eg. tonsil, endometrium, heart and skeletal muscle). Perinuclear staining pattern. Not present in skeletal muscle from patient with Emery-Dreifuss muscular dystrophy. Storage and Stability: Lyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Catalogue number: E2257-01E
Reactivities: Human
Hosts: Mouse
Applications: Immunohistochemistry, Western Blot
Size: 1ml
Form: Supplied as a lyophilized powder in 15mM sodium azide. Reconstitute with 1ml dH2O.
P type: Mab
Isotype: IgG1
Purity: Supernatant
Alternative names: Emery-Dreifuss Muscular Dystrophy
References: 1.Nagano A, Koga R, Ogawa M, et al.. Emerin deciciency at the nuclear membrane in patients with Emery-Dreifuss muscular dystrophy. Nature Genetics. 12:254-259 (1996). 2.Bione S, Small K, Aksmanovic V, et al.. Identification of new mutations in the Emery-Dreifuss muscular dystrophy gene and evidence for genetic het- erogeneity of the disease. Human Molecular Genetics. 4:1859-1863 (1995). 3.Metzenberg A and Gitschier J. New mutations in the gene encoding emerin that are responsible for emery-dreifuss muscular-dystrophy. American Journal of Human Genetics. 57:1271 (1995). 4.Nigro V, Bruni P, Ciccodicola P, et al.. SSCP detection of novel mutations in patients with Emery-Dreifuss muscular dystrophy: Definition of a small c- terminal region required for emerin function. Human Molecular Genetics. 4:2003-2004 (1995). 5.Bione S, Maestrini E, Rivella S, et al.. Identification of a novel X-linked gene responsible for Emery-Dreifuss muscular dystrophy. Nature Genetics. 8:323-327 (1994).
Additional info: Recognizes Emerin. Species Crossreactivity: Human.

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