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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UBR2
ubiquitin protein ligase E3 component n-recognin 2
Chromosome 6 · 6p21.1
NCBI Gene: 23304Ensembl: ENSG00000024048.12HGNC: HGNC:21289UniProt: B3KXG6
89PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin protein ligase activityL-leucine bindingnegative regulation of TOR signalingAbnormality of the skeletal systemfrozen shoulderneoplasmcancer
✦AI Summary

UBR2 is an E3 ubiquitin ligase functioning as a component of the N-end rule pathway, recognizing proteins bearing destabilizing N-terminal residues (N-degrons) and marking them for ubiquitin-dependent proteasomal degradation 1. UBR2 recognizes both type-1 N-degrons (positively charged residues) and type-2 N-degrons (bulky hydrophobic residues), with activity regulated by N-terminal acetylation status. Beyond canonical N-degron recognition, UBR2 exhibits diverse cellular functions: it negatively regulates mTOR signaling through leucine binding, controlling cell growth 2, and positively regulates T-cell receptor signaling by catalyzing Lys63-linked ubiquitination of the kinase Lck, promoting inflammatory responses 3. UBR2 is dysregulated in disease contexts. In primary ovarian insufficiency, UBR2 mutations impair meiotic processes critical for fertility 4. In pancreatic cancer cachexia, UBR2 induction in muscle tissue promotes myofibrillar protein degradation and muscle wasting 5. Conversely, constitutively active UBR2 ameliorates hepatic steatosis by degrading lipid droplet proteins 6. UBR2 function is tightly controlled: DUSP22 phosphatase inactivates UBR2 through Lys48-linked ubiquitination, dampening inflammation 3. Complete loss of both UBR1 and UBR2 causes embryonic lethality with neurogenesis and cardiovascular defects 7, indicating essential roles in development.

Sources cited
1
UBR1 and UBR2 are sequelogous E3 ubiquitin ligases that mediate Arg/N-degron pathway targeting and recognition of N-terminal destabilizing residues
PMID: 32692156
2
UBR2 regulates proteasomal protein degradation and promotes cell survival under amino acid starvation through interaction with SOD2
PMID: 40131931
3
UBR2 induces Lys63-linked ubiquitination of Lck to promote T-cell receptor signaling and proinflammatory cytokine expression; DUSP22 phosphatase controls UBR2 via Lys48-linked ubiquitination
PMID: 38225265
4
UBR2 mutations are associated with primary ovarian insufficiency, a disorder affecting meiosis and follicle development
PMID: 34794894
5
UBR2 expression is induced in muscle tissue downstream of pancreatic cancer-derived extracellular vesicles, promoting myofibrillar protein degradation and muscle wasting
PMID: 30342032
6
Constitutively active UBR2 ameliorates hepatic steatosis by ubiquitinating and degrading lipid droplet-stabilizing proteins
PMID: 35245436
7
Double UBR1-/-UBR2-/- embryos die at midgestation with defects in neurogenesis and cardiovascular development, indicating essential non-redundant functions
PMID: 16606826
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.28Weak
frozen shoulderOpen Targets
0.22Weak
neoplasmOpen Targets
0.09Suggestive
cancerOpen Targets
0.08Suggestive
triple-negative breast cancerOpen Targets
0.08Suggestive
azoospermiaOpen Targets
0.07Suggestive
neural tube defects, folate-sensitiveOpen Targets
0.06Suggestive
anencephaly 1Open Targets
0.06Suggestive
Isolated anencephaly/exencephalyOpen Targets
0.06Suggestive
Spina bifida - hypospadiasOpen Targets
0.06Suggestive
spina bifida-hypospadias syndromeOpen Targets
0.06Suggestive
partial chromosome Y deletionOpen Targets
0.05Suggestive
spermatogenic failure 25Open Targets
0.04Suggestive
spermatogenic failure 84Open Targets
0.04Suggestive
spermatogenic failure 93Open Targets
0.04Suggestive
spermatogenic failure 65Open Targets
0.04Suggestive
spermatogenic failure 54Open Targets
0.04Suggestive
Isolated follicle stimulating hormone deficiencyOpen Targets
0.04Suggestive
spermatogenic failure 56Open Targets
0.04Suggestive
spermatogenic failure 92Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UBE2AProtein interaction98%UBE2BProtein interaction98%UBR5Protein interaction93%UBR4Protein interaction89%TRIP12Protein interaction84%RECQL4Protein interaction84%
Tissue Expression6 tissues
Lung
100%
Heart
90%
Bone Marrow
85%
Ovary
79%
Liver
76%
Brain
60%
Gene Interaction Network
Click a node to explore
UBR2UBE2AUBE2BUBR5UBR4TRIP12RECQL4
PROTEIN STRUCTURE
Preparing viewer…
PDB5TDA · 0.79 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.47Moderately Constrained
pLIⓘ
0.97Intolerant
Observed/Expected LoF0.39 [0.32–0.47]
RankingsWhere UBR2 stands among ~20K protein-coding genes
  • #5,390of 20,598
    Most Researched89
  • #2,715of 17,882
    Most Constrained (LOEUF)0.47 · top quartile
Genes detectedUBR2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Genetics of ovarian insufficiency and defects of folliculogenesis.
PMID: 34794894
Best Pract Res Clin Endocrinol Metab · 2022
1.00
2
Amelioration of hepatic steatosis by dietary essential amino acid-induced ubiquitination.
PMID: 35245436
Mol Cell · 2022
0.90
3
The phosphatase DUSP22 inhibits UBR2-mediated K63-ubiquitination and activation of Lck downstream of TCR signalling.
PMID: 38225265
Nat Commun · 2024
0.80
4
ZIP4 Promotes Muscle Wasting and Cachexia in Mice With Orthotopic Pancreatic Tumors by Stimulating RAB27B-Regulated Release of Extracellular Vesicles From Cancer Cells.
PMID: 30342032
Gastroenterology · 2019
0.70
5
Exploration of novel mechanisms of azole resistance in
PMID: 39480072
Antimicrob Agents Chemother · 2024
0.60