Malin Monoclonal Antibody
Catalog no.: 56554 (clone S85-18)
Format: 100ug (1mg/ml) Protein G-purified antibody in PBS, pH 7.4, 0.1% sodium azide, 50% glycerol.
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. 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. 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 interacts with and poly-ubiquitinates 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.
Antigen: Fusion protein corresponding to aa 2-125 (N-terminus encompassing RING domain) of human Malin. This sequence is 86% identical to rat and 77% identical to mouse.
Accession nos.: NP_940988.2, Q6VVB1
Gene Id: 378884 Host Species: Mouse Antibody Class: IgG1
Specificity: This antibody recognizes human Malin.
Immunoblotting: use at 1-5ug/ml. A band of ~42kDa is detected.
Immunofluorescence: use at 10ug/ml.
These are recommended concentrations. Endusers should determine optimal concentrations for their application.
Dilute in PBS or medium that is identical to that used in the assay system.
STORAGE AND STABILITY
This product is stable for at least one (1) year at -20°C.
For in vitro investigational use only. Not intended for therapeutic or diagnostic procedures.