InnateDB: Systems Biology of the Innate Immune Response

Mus musculus Protein: Nhlrc1

Summary

InnateDB Protein

IDBP-148922.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

Nhlrc1

Protein Name

NHL repeat containing 1

Synonyms

AI505271; B230309E09Rik; EPM2B;

Species

Mus musculus

Ensembl Protein

ENSMUSP00000054990

InnateDB Gene

IDBG-148920 (Nhlrc1)

Protein Structure

UniProt Annotation

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. {ECO:0000269PubMed:19036738, ECO:0000269PubMed:21077101, ECO:0000269PubMed:22186026, ECO:0000269PubMed:22669944}.

Subcellular Localization

Endoplasmic reticulum {ECO:0000250}. Nucleus {ECO:0000250}. Note=Localizes at the endoplasmic reticulum and, to a lesser extent, in the nucleus. {ECO:0000250}.

Disease Associations

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.
They are also associated with 14 interaction(s) predicted by orthology.

Experimentally validated
Total	4 [view]
Protein-Protein	4 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	14 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004842	ubiquitin-protein transferase activity
GO:0005515	protein binding
GO:0008270	zinc ion binding
GO:0016874	ligase activity

Biological Process

GO:0000209	protein polyubiquitination
GO:0006914	autophagy
GO:0031398	positive regulation of protein ubiquitination
GO:0043161	proteasome-mediated ubiquitin-dependent protein catabolic process