Malin (E3 Ubiquitin-Protein Ligase NHLRC1, NHLRC 1, NHL Repeat Containing 1, EPM2A, EPM2B, NHL Repeat Containing Protein 1)

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£626.00

SKU:: 384898
Anwendung:: WB
Reinheit:: Purified by Protein G affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Mouse
Immunogen:: Fusion protein corresponding to aa2-125 (N-terminal encompassing RING domain) of human Malin. Species sequence homology: rat (86%), mouse (77%)
Klon:: S85-18
Format:: Supplied as a liquid in PBS, pH 7.4, 0.09% sodium azide, 50% glycerol.
Weitere Details:: Progressive myoclonic epilepsy type 2 (EPM2), also called Lafora disease, is an autosomal recessive disease characterized by grand mal seizures and/or myoclonus at about 15 years of age. Rapid and severe mental deterioration follows, often with psychotic features. Survival is less than 10 years after onset. Starch-like, endoplasmic reticulum-associated polyglucosans, called Lafora bodies, can be observed in brain, muscle, liver and heart. One cause of Lafora disease is due to mutations in NHLRC1, the gene encoding Malin. Forty-nine different mutations in NHLRC1 have been shown to cause EPM2. Malin, also called NHL repeat-containing protein 1, is a single subunit E3 ubiquitin ligase, containing 6 NHL repeats and 1 RING-type zinc finger. Malin’s RING domain is responsible for its ability to mediate ubiquitination. Malin interacts with and polyubiquitinates Laforin, a protein also implicated in EPM2. Malin localizes to the endoplasmic reticulum and, to a lesser extent, in the nucleus. Malin is expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas. Applications: Suitable for use in Western Blot, Immunocytochemistry/Immunofluorescence, Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1:1000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Versandbedingungen:: Blue Ice