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Target intelligence / Profile preview
ER degradation-enhancing alpha-mannosidase-like protein 2 (EDEM2)
- Target
- EDEM2
- Molecular classification
- Enzyme, Mannosidase homolog, Component of the endoplasmic reticulum-associated degradation (ERAD) pathway
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Overview
ER degradation-enhancing alpha-mannosidase-like protein 2 (EDEM2) is an enzyme of the endoplasmic reticulum that initiates the degradation of misfolded glycoproteins via the ER-associated degradation (ERAD) pathway by catalyzing the first mannose trimming step (conversion of Man9GlcNAc2 to Man8GlcNAc2) on glycoprotein N-glycans. EDEM2 forms a disulfide-bonded complex with TXNDC11, which is essential for its enzymatic activity, and functions as a central sensor and effector in ER quality control, facilitating the recognition and targeting of terminally misfolded glycoproteins for retrotranslocation and proteasomal degradation. EDEM2 is upregulated during ER stress and plays a critical role in cellular protein homeostasis.
- Other names
- EDEM2C20orf31C20orf49FLJ10783UNQ573/PRO1135bA4204.1ER degradation enhancer, mannosidase alpha-like 2ER degradation-enhancing mannosidase-like protein 2
02
Biological functions
Protein quality controlER-associated degradation (ERAD)Mannose trimming from misfolded glycoproteinsMaintenance of proteostasisResponse to ER stress
03
Disease associations
Congenital disorder of glycosylation, Type InPossibly implicated in neurodegeneration, proteostasis diseasesAltered plasma protein C regulation (potential link to coagulation disorders)
04
Safety considerations
Disruption of EDEM2 or its ERAD pathway can lead to accumulation of misfolded proteins and ER stress, which are associated with cell death and diseases such as neurodegeneration or congenital disorders.
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