NHLRC1 Polyclonal Antibody | Invitrogen PA5-121023 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

Invitrogen

other brands :

NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience

product type :

antibody

product name :

NHLRC1 Polyclonal Antibody

catalog :

PA5-121023

quantity :

100 uL

price :

US 460.00

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, immunohistochemistry - paraffin section

product information

Product Type :

Antibody

Product Name :

NHLRC1 Polyclonal Antibody

Catalog # :

PA5-121023

Quantity :

100 uL

Price :

US 460.00

Clonality :

Polyclonal

Purity :

Affinity Chromatography

Host :

Rabbit

Reactivity :

Human, Mouse, Rat

Applications :

ELISA: 1 ug/mL, Immunohistochemistry (Paraffin): 1:50-1:200, Western Blot: 1:2,000-1:7,000

Species :

Human, Mouse, Rat

Isotype :

IgG

Storage :

-20 C, Avoid Freeze/Thaw Cycles

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.

Immunogen :

Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human NHLRC1 (NP_9409882)

Format :

Liquid

Applications w/Dilutions :

ELISA: 1 ug/mL, Immunohistochemistry (Paraffin): 1:50-1:200, Western Blot: 1:2,000-1:7,000

Aliases :

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