Anti-NHLRC1 Antibody

$99.00$319.00

Reactivity: Human,Rat
Applications: ELISA,WB,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-NHLRC1 Antibody (CSB-PA740936ESR2HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

E3 ubiquitin-protein ligase NHLRC1 Antibody,Malin Antibody,NHL repeat-containing protein 1 Antibody,NHLRC1 Antibody,EPM2B Antibody

More alternative names for the antibody
bA204B7.2 antibody|E3 ubiquitin-protein ligase NHLRC1 antibody|EPM2A antibody|EPM2B antibody|EPM2B gene antibody|Malin antibody|MGC119262 antibody|MGC119264 antibody|MGC119265 antibody|NHL repeat containing 1 antibody|NHL repeat containing E3 ubiquitin protein ligase 1 antibody|NHL repeat containing protein 1 antibody|NHL repeat-containing protein 1 antibody|NHL repeat-containing protein 1 gene antibody|NHLC1_HUMAN antibody|NHLRC 1 antibody|Nhlrc1 antibody
Anti-NHLRC1 antibody [S8518] (ab186261)
Close sc-293401|sc-67360|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-NHLRC1 Antibody

Catalogue No.

CSB-PA740936ESR2HU

Reactivity

Human, Rat

Immunogen

Recombinant human E3 ubiquitin-protein ligase NHLRC1 protein (1-280AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

42 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:378884( Human), Entrez Gene:364682( Rat), Omim:608072( Human), SwissProt:Q6VVB1( Human), SwissProt:Q6IMG5( Rat), Unigene:348351( Human), Unigene:103777( Rat)

Protein 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. .

Protein tissue specificity

Expressed in brain, cerebellum, spinal cord, medulla, heart, liver, skeletal muscle and pancreas. .

Involvement in disease

Epilepsy, progressive myoclonic 2 (EPM2) [MIM:254780]: An autosomal recessive and severe form of adolescent-onset progressive epilepsy. Typically, as seizures increase in frequency, cognitive function declines towards dementia, and affected individuals die usually within 10 years after onset. EPM2 occurs worldwide, but it is particularly common in the mediterranean countries of southern Europe and northern Africa, in southern India and in the Middle East. At the cellular level, it is characterized by accumulation of starch-like polyglucosans called Lafora bodies (LBs) that are most abundant in organs with the highest glucose metabolism: brain, heart, liver and skeletal muscle. Among other conditions involving polyglucosans, EPM2 is unique in that the inclusions are in neuronal dendrites but not axons and the forming polyglucosan fibrils are associated with the endoplasmic reticulum. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

The RING domain is essential for ubiquitin E3 ligase activity.

Protein cellular localization

Endoplasmic reticulum. Nucleus. Note=Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus.

Research area

All research areas>Signaling Intermediates>Malin
(View all antibody categories related to Signaling Intermediates)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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