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GeneE
4 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ARK2C
arkadia (RNF111) C-terminal like ring finger ubiquitin ligase 2C
Chromosome 18 · 18q21.1
NCBI Gene: 494470Ensembl: ENSG00000141622.15HGNC: HGNC:31696UniProt: A0A059NXK6
15PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin protein ligase activitymotor neuron axon guidancepositive regulation of BMP signaling pathwayprotozoa infectious diseaseesophageal ulcerself-injurious ideationenteritis
✦AI Summary

ARK2C is an E3 ubiquitin ligase that functions as a critical regulator of motor axon elongation during nervous system development 1. As a RING-domain E3 ligase, ARK2C catalyzes ubiquitin chain assembly through a distinctive mechanism in which its RING domain directly binds free ubiquitin, coordinating with E2-conjugated ubiquitin to stabilize the catalytic complex and enhance ubiquitin transfer efficiency 2. This unique ubiquitin-dependent activation distinguishes ARK2C from many other RING ligases 3. Functionally, ARK2C promotes motor axon extension by enhancing BMP-SMAD signaling; it mediates ubiquitination and degradation of SMAD inhibitors (SMAD6, SMAD7, SKI, and SNON), thereby potentiating phosphorylated SMAD1/5/8 transcriptional responses 1. ARK2C recruits multiple E2 conjugating enzymes with context-specific selectivity, enabling assembly of distinct ubiquitin chain types—both degradative and non-degradative—depending on the E2 partner and substrate 4. Loss of ARK2C in mice causes severe motor innervation defects including reduced motor axon extension and impaired neuromuscular junction formation, resulting in perinatal lethality, underscoring its essential developmental role 1. These findings establish ARK2C as a specialized E3 ligase linking ubiquitin-dependent proteolysis to BMP signaling control in motor neuron development.

Sources cited
1
ARK2C expression in nervous system, loss causes motor innervation defects, enhances BMP-SMAD signaling to promote motor axon extension
PMID: 23610558
2
ARK2C recruits multiple E2 enzymes and promotes substrate ubiquitylation with context-specific chain types
PMID: 40451499
3
ARK2C RING domain binds free ubiquitin and stabilizes E2-ubiquitin conjugate in catalytically active conformation
PMID: 26656854
4
ARK2C adopts alternative ubiquitin-dependent mechanism for ubiquitin transfer activation with high-affinity binding to ubiquitin
PMID: 33086510
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
esophageal ulcerOpen Targets
0.31Weak
protozoa infectious diseaseOpen Targets
0.31Weak
self-injurious ideationOpen Targets
0.30Weak
enteritisOpen Targets
0.30Weak
alcohol drinkingOpen Targets
0.28Weak
vertebral joint diseaseOpen Targets
0.07Suggestive
deficiency anemiaOpen Targets
0.04Suggestive
Down syndromeOpen Targets
0.03Suggestive
ataxia telangiectasiaOpen Targets
0.01Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
aleutian mink diseaseOpen Targets
0.01Suggestive
Tetralogy of FallotOpen Targets
0.01Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
astrocytomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
posterior cortical atrophyOpen Targets
0.01Suggestive
ulcerative colitisOpen Targets
0.01Suggestive
uterine leiomyosarcomaOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UBE2D2Protein interaction93%UBA52Protein interaction83%UBCProtein interaction71%KCPShared pathway50%ELAPOR2Shared pathway33%SCUBE3Shared pathway25%
Tissue Expression6 tissues
Brain
100%
Liver
60%
Heart
20%
Bone Marrow
12%
Lung
6%
Ovary
1%
Gene Interaction Network
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ARK2CUBE2D2UBA52UBCKCPELAPOR2SCUBE3
PROTEIN STRUCTURE
Preparing viewer…
PDB5D0I · 1.90 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.50Moderately Constrained
pLIⓘ
0.98Intolerant
Observed/Expected LoF0.31 [0.20–0.50]
RankingsWhere ARK2C stands among ~20K protein-coding genes
  • #15,487of 20,598
    Most Researched15
  • #2,990of 17,882
    Most Constrained (LOEUF)0.50 · top quartile
Genes detectedARK2C
Sources retrieved4 papers
Response time—
📄 Sources
4
1
Arkadia and Ark2C Promote Substrate Ubiquitylation with Multiple E2 Enzymes.
PMID: 40451499
J Mol Biol · 2025
1.00
2
Rnf165/Ark2C enhances BMP-Smad signaling to mediate motor axon extension.
PMID: 23610558
PLoS Biol · 2013
0.75
3
Assessing the Direct Binding of Ark-Like E3 RING Ligases to Ubiquitin and Its Implication on Their Protein Interaction Network.
PMID: 33086510
Molecules · 2020
0.50
4
Secondary ubiquitin-RING docking enhances Arkadia and Ark2C E3 ligase activity.
PMID: 26656854
Nat Struct Mol Biol · 2016
0.25