NHLRC1 Polyclonal Antibody, Invitrogen™
Manufacturer: Thermo Scientific
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | PIPA5113015-Each-of-1 | In Stock | ₹ 45,078.50 |
PIPA5113015 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 45,078.50
GST (18%): ₹ 8,114.13
Total Price: ₹ 53,192.63
Antigen
NHLRC1
Classification
Polyclonal
Conjugate
Unconjugated
Gene
NHLRC1
Gene Alias
AI505271; B230309E09Rik; bA204B7.2; E3 ubiquitin-protein ligase NHLRC1; EPM2A; EPM2B; Malin; NHL repeat containing 1; NHL repeat containing E3 ubiquitin protein ligase 1; NHL repeat-containing protein 1; NHLRC1; RING-type E3 ubiquitin transferase NHLRC1
Host Species
Rabbit
Purification Method
Antigen affinity chromatography
Regulatory Status
RUO
Gene ID (Entrez)
364682, 378884
Content And Storage
-20°C or -80°C if preferred
Form
Liquid
Applications
ELISA, Immunohistochemistry (Paraffin), Western Blot
Concentration
3.63 mg/mL
Formulation
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene Accession No.
Q6IMG5, Q6VVB1
Gene Symbols
NHLRC1
Immunogen
Recombinant Human E3 ubiquitin-protein ligase NHLRC1 protein (1-280AA)
Quantity
100 μL
Primary or Secondary
Primary
Target Species
Human, Rat
Product Type
Antibody
Isotype
IgG
Related Products
Description
- 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.