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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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UBE2J2
ubiquitin conjugating enzyme E2 J2
Chromosome 1 · 1p36.33
NCBI Gene: 118424Ensembl: ENSG00000160087.21HGNC: HGNC:19268UniProt: A6NGS0
41PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin protein ligase bindingubiquitin conjugating enzyme activityubiquitin-dependent protein catabolic processhemoglobin D diseasedehydrated hereditary stomatocytosisAdult-onset autosomal recessive sideroblastic anemiamicrocytic anemia with liver iron overload
✦AI Summary

UBE2J2 is an ER-membrane-anchored ubiquitin-conjugating enzyme (E2) that catalyzes the covalent attachment of ubiquitin to target proteins, with particular importance in ER-associated degradation (ERAD) 1. Unlike most E2 enzymes that exclusively catalyze lysine ubiquitination, UBE2J2 possesses unique structural features enabling noncanonical serine ubiquitination through a two-layered mechanism involving active site rearrangement and a conserved histidine that activates substrate serines 2. UBE2J2 functions selectively in degrading specific misfolded ER proteins; it partners with the E3 ligase MARCH6 for cholesterol-stimulated degradation of squalene monooxygenase (SQLE), distinct from UBE2G2's role in HMGCR degradation 3. Critically, UBE2J2 acts as a lipid sensor, responding to membrane lipid saturation changes: loose lipid packing impairs ubiquitin loading and activity, while tight packing promotes its active conformation 1. Beyond ERAD, UBE2J2 participates in EVI/WLS ubiquitylation for WNT pathway regulation 4 and cooperates with GATOR2 to catalyze Lys-6-linked ubiquitination of NPRL2. Dysregulation of UBE2J2 has clinical relevance: it enhances venetoclax sensitivity in acute myeloid leukemia when depleted alongside MARCH5 5, and elevated UBE2J2 expression associates with poor colorectal cancer prognosis 6. Intriguingly, UBE2J2 itself undergoes proteasomal degradation, suggesting its steady-state levels regulate ER protein quality control 7.

Sources cited
1
UBE2J2 is a membrane-anchored E2 that senses lipid packing in the ER and modulates its activity accordingly; functions in ERAD with E3 ligases RNF145, MARCHF6, and RNF139
PMID: 41068091
2
UBE2J2 catalyzes noncanonical serine ubiquitination through active site rearrangement and histidine-mediated general base catalysis
PMID: 39533056
3
UBE2J2 is the primary E2 enzyme essential for MARCH6-dependent, cholesterol-stimulated degradation of SQLE
PMID: 30658189
4
UBE2J2 mediates K11, K48, and K63-linked ubiquitylation of EVI/WLS in ERAD regulation of the WNT secretory pathway
PMID: 34406391
5
UBE2J2 depletion sensitizes AML cells to venetoclax in cooperation with MARCH5 E3 ligase
PMID: 36973350
6
Elevated UBE2J2 expression is part of a pyroptosis-related gene signature predicting poor prognosis in colorectal cancer
PMID: 39155627
7
UBE2J2 itself is an unstable protein subject to proteasomal degradation in a manner dependent on its ER localization and enzymatic activity
PMID: 25083800
Disease Associationsⓘ20
hemoglobin D diseaseOpen Targets
0.05Suggestive
dehydrated hereditary stomatocytosisOpen Targets
0.05Suggestive
Adult-onset autosomal recessive sideroblastic anemiaOpen Targets
0.04Suggestive
microcytic anemia with liver iron overloadOpen Targets
0.04Suggestive
hemoglobin E diseaseOpen Targets
0.04Suggestive
overhydrated hereditary stomatocytosisOpen Targets
0.04Suggestive
hereditary elliptocytosisOpen Targets
0.04Suggestive
sideroblastic anemia 3Open Targets
0.04Suggestive
Blackfan-Diamond anemiaOpen Targets
0.03Suggestive
fibular hemimeliaOpen Targets
0.03Suggestive
ring chromosome YOpen Targets
0.03Suggestive
46,XY partial gonadal dysgenesisOpen Targets
0.03Suggestive
aromatase deficiencyOpen Targets
0.03Suggestive
Weismann-Netter syndromeOpen Targets
0.03Suggestive
ghosal hematodiaphyseal dysplasiaOpen Targets
0.03Suggestive
46,XY disorder of sex development due to isolated 17,20 lyase deficiencyOpen Targets
0.03Suggestive
Camurati-Engelmann diseaseOpen Targets
0.03Suggestive
bone marrow failure syndrome 6Open Targets
0.03Suggestive
tibial hemimeliaOpen Targets
0.03Suggestive
hypotonia-cystinuria syndromeOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VCPProtein interaction100%UBCProtein interaction97%MARCHF6Protein interaction90%SELENOSProtein interaction90%DERL1Protein interaction90%NECAP1Protein interaction88%
Tissue Expression6 tissues
Liver
100%
Lung
81%
Ovary
70%
Bone Marrow
46%
Heart
45%
Brain
42%
Gene Interaction Network
Click a node to explore
UBE2J2VCPUBCMARCHF6SELENOSDERL1NECAP1
PROTEIN STRUCTURE
Preparing viewer…
PDB2F4W · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.31Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.10 [0.04–0.31]
RankingsWhere UBE2J2 stands among ~20K protein-coding genes
  • #10,108of 20,598
    Most Researched41
  • #1,199of 17,882
    Most Constrained (LOEUF)0.31 · top 10%
Genes detectedUBE2J2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Targeting a mitochondrial E3 ubiquitin ligase complex to overcome AML cell-intrinsic Venetoclax resistance.
PMID: 36973350
Leukemia · 2023
1.00
2
Determinants of chemoselectivity in ubiquitination by the J2 family of ubiquitin-conjugating enzymes.
PMID: 39533056
EMBO J · 2024
0.90
3
UBE2J2 sensitizes the ERAD ubiquitination cascade to changes in membrane lipid saturation.
PMID: 41068091
Nat Commun · 2025
0.80
4
The UBE2J2/UBE2K-MARCH5 ubiquitination machinery regulates apoptosis in response to venetoclax in acute myeloid leukemia.
PMID: 38366087
Leukemia · 2024
0.70
5
A Pyroptosis-Related Gene Signature Predicts Prognosis and Tumor Immune Microenvironment in Colorectal Cancer.
PMID: 39155627
Technol Cancer Res Treat · 2024
0.60