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Anti-NHLRC1 Polyclonal Antibody
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Catalog No. TMAB-09469
Anti-NHLRC1 Polyclonal Antibody is a Rabbit antibody targeting NHLRC1. Anti-NHLRC1 Polyclonal Antibody can be used in WB.
Anti-NHLRC1 Polyclonal Antibody
Anti-NHLRC1 Polyclonal Antibody
😃Good
Catalog No. TMAB-09469
Anti-NHLRC1 Polyclonal Antibody is a Rabbit antibody targeting NHLRC1. Anti-NHLRC1 Polyclonal Antibody can be used in WB.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $221 | 7-10 days | 7-10 days | |
| 100 μL | $372 | 7-10 days | 7-10 days | |
| 200 μL | $527 | 7-10 days | 7-10 days |
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In Stock Estimated shipping dateUSA Warehouse[1-2 days] Global Warehouse[5-7 days]
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Product Introduction
Bioactivity
Antigen Details
Chemical Properties
| Description | Anti-NHLRC1 Polyclonal Antibody is a Rabbit antibody targeting NHLRC1. Anti-NHLRC1 Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| Reactivity | Rat (predicted:Human,Mouse,Rabbit) |
| Application | |
| Recommended Dose | WB: 1:500-2000 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | Endoplasmic reticulum. Nucleus. Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus. |
| Tissue Specificity | Expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research Background | 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. |
| Immunogen | KLH conjugated synthetic peptide: human NHLRC1 |
| Antigen Species | Human |
| Gene Name | NHLRC1 |
| Gene ID | |
| Protein Name | E3 ubiquitin-protein ligase NHLRC1 |
| Uniprot ID | |
| Function | E3 ubiquitin-protein ligase which 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 PPP1R3C/PTG in a laforin-dependent manner, and targets it for proteasome-dependent degradation and this degradation decreases glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation. |
| Molecular Weight | Theoretical: 42 kDa. |
Stability & Storage
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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