NHLRC1 / FITC / S85-18

Product Details
Supplier Biorbyt
Catalog #: orb150580-100ug (View supplier product page)
Size 100 μg
Price $561.00
Antigen NHLRC1
Clone S85-18
Host Mouse
Isotype IgG1
Conjugate FITC
Target Species Human
Applications WB, IF, ICC
Description Mouse monoclonal to Malin (FITC). 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. Malins 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..
About NHLRC1 and FITC
NHLRC1 The protein encoded by this gene is a single subunit E3 ubiquitin ligase. Laforin is polyubiquitinated by the encoded protein. Defects in this intronless gene lead to an accumulation of laforin and onset of Lafora disease, also known as progressive myoclonic epilepsy type 2 (EPM2).[provided by RefSeq, Mar 2010]
FITC Fluorescein isothiocyanate (FITC) has an excitation peak at 495 nm and an emission peak at 519 nm. The name FITC is a misnomer in that the isothiocyanate is a reactive form of this dye. Once FITC is conjugated to an antibody, it is simply Fluorescein conjugated. FITC is one of the most widely used dyes for fluorescent applications, therefore most instruments come standard with a 488 nm laser and FITC filter set up. FITC is commonly conjugated to secondary antibodies and used in applications such as flow cytometry, immunocytochemistry, and immunohistochemistry. FITC is relatively dim, sensitive to photobleaching and it is susceptible to changes is pH. There are better performing alternatives to FITC, like Vio®Bright 515, Alexa Fluor™ 488, iFluor® 488, CF®488A and DY-488. FITC is a long-time generic dye with no sole manufacturer or trademark.
Citations
Additional
Sources
Reviews & Ratings
Submit a Review

FITC Excitation and Emission Spectra