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

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.\n\nApplications: \nSuitable for use in Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:250-1:500.\nImmunohistochemistry (Frozen): 1:20-1:40 for 1 hr. at RT\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nWestern Blot: Sup T cells\nImmunohistochemistry: 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.\n\nStorage and Stability:\nLyophilized 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.

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SPECIFICATIONS

Catalog Number

E2257-01E

Size

1ml

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a lyophilized powder in 15mM sodium azide. Reconstitute with 1ml dH2O.

P Type

Mab

Purity

Supernatant

Isotype

IgG1

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.

Alternative Names

Emery-Dreifuss Muscular Dystrophy