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NHLRC1 Antibody (Center) Blocking peptide

Synthetic peptide

Product Information
Primary Accession Q6VVB1
Clone Names 100324041
Peptide ID 100324041
Additional Information
Gene ID 378884
Other Names E3 ubiquitin-protein ligase NHLRC1, 632-, Malin, NHL repeat-containing protein 1, NHLRC1, EPM2B
Target/Specificity The synthetic peptide sequence used to generate the antibody AP13383c was selected from the Center region of NHLRC1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Format Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Synonyms EPM2B
Function E3 ubiquitin-protein ligase. Together with the phosphatase EPM2A/laforin, appears to be involved in the clearance of toxic polyglucosan and protein aggregates via multiple pathways. In complex with EPM2A/laforin and HSP70, suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates the glycogen-targeting protein phosphatase subunits PPP1R3C/PTG and PPP1R3D in a laforin-dependent manner and targets them for proteasome-dependent degradation, thus decreasing glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation. Also promotes proteasome-independent protein degradation through the macroautophagy pathway.
Cellular Location Endoplasmic reticulum. Nucleus. Note=Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus
Tissue Location Expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas
Research Areas
Citations (0)

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The protein encoded by this gene is a single subunit E3ubiquitin ligase. Laforin is polyubiquitinated by the encodedprotein. Defects in this intronless gene lead to an accumulation oflaforin and onset of Lafora disease, also known as progressivemyoclonic epilepsy type 2 (EPM2).


Moreno, D., et al. Mol. Biol. Cell 21(15):2578-2588(2010)Rao, S.N., et al. J. Biol. Chem. 285(2):1404-1413(2010)Traore, M., et al. Neurogenetics 10(4):319-323(2009)Singh, S., et al. Hum. Mutat. 30(5):715-723(2009)Vernia, S., et al. PLoS ONE 4 (6), E5907 (2009) :

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Cat# BP13383c
Availability: Back Order
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