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Anti-NHLRC1 Polyclonal Antibody

Catalog No. TMAB-09469 Copy Product Info
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Anti-NHLRC1 Polyclonal Antibody is a Rabbit antibody targeting NHLRC1. Anti-NHLRC1 Polyclonal Antibody can be used in WB.

Anti-NHLRC1 Polyclonal Antibody

Copy Product Info
🥰Excellent
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
Pack SizePriceUSA StockGlobal StockQuantity
50 μL$2217-10 days7-10 days
100 μL$3727-10 days7-10 days
200 μL$5277-10 days7-10 days
For In stock only · Estimated delivery: USA Stock (1-2 days) Global Stock (5-7 days)
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For research use only—not for human use. No sales to individuals. Use as intended only.
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Product Introduction

Bioactivity
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)
Verified Activity
Sample: liver (Rat) Lysate at 40 μg
Primary: Anti-NHLRC1 (TMAB-09469) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 42 kD
Observed band size: 52 kD
Application
Recommended Dose
WB: 1:500-2000
Antibody Type
Polyclonal
Host SpeciesRabbit
Subcellular LocalizationEndoplasmic reticulum. Nucleus. Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus.
Tissue SpecificityExpressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas.
ConstructionPolyclonal Antibody
PurificationProtein A purified
AppearanceLiquid
Formulation0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Concentration1mg/ml
Research BackgroundProgressive 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.
Antigen Details
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.
Chemical Properties
Molecular WeightTheoretical: 42 kDa.
Stability & Storage
Stability & StorageStore at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles.
TransportShipping with blue ice.

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