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Emerin Muscle Disease Antibodies

Emerin

Human skeletal muscle: immunohistochemical staining for emerin using  NCL-EMERIN. Note perinuclear staining of all cell nuclei. Paraffin section.
Human skeletal muscle: immunohistochemical staining for emerin using NCL-EMERIN. Note perinuclear staining of all cell nuclei. Paraffin section.

Antigen 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 postcervical 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.


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Code Name Configuration Use Datasheet MSDS Qty
EMERIN-CE-S 0.1ml NCL-EMERIN Lyophilised Concentrated Monoclonal Antibody P(HIER) Info MSDS
EMERIN-CE 1ml NCL-EMERIN Lyophilised Concentrated Monoclonal Antibody F, P(HIER) Info MSDS
EMERIN 1ml NCL-EMERIN Lyophilised Concentrated Monoclonal Antibody F, P(HIER), W Info MSDS