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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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FEM1C
fem-1 homolog C
Chromosome 5 · 5q22.3
NCBI Gene: 56929Ensembl: ENSG00000145780.9HGNC: HGNC:16933UniProt: Q96JP0
26PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingubiquitin-like ligase-substrate adaptor activityproteasome-mediated ubiquitin-dependent protein catabolic processubiquitin-dependent protein catabolic process via the C-end degron rule pathwayAtaxiacerebellar ataxiaNeurodevelopmental disorderneurodegenerative disease
✦AI Summary

FEM1C is a substrate recognition component of the Cul2-RING E3 ubiquitin ligase complex that mediates protein degradation through the DesCEND (destruction via C-end degrons) pathway 1. The protein specifically recognizes C-degrons—degradation signals at protein C-termini—with particular specificity for arginine residues at the extreme C-terminus, targeting substrates with -Lys/Arg-Xaa-Arg and -Lys/Arg-Xaa-Xaa-Arg motifs 2. FEM1C utilizes a semi-open binding pocket to capture C-terminal arginines, with the extreme C-terminal residue serving as the major structural determinant for recognition 2. Known substrates include SIL1, OR51B2, truncated MSRB1/SEPX1 selenoproteins produced by failed UGA/Sec decoding, and SLBP (Stem-Loop Binding Protein), which is regulated across the cell cycle 3. FEM1C is evolutionarily conserved from C. elegans to vertebrates 4. Clinically, de novo FEM1C variants have been associated with neurodevelopmental disorders characterized by developmental delay, pyramidal signs, and limb ataxia, likely through impaired substrate binding 5. Additionally, FEM1C expression levels show predictive capacity for liver transplantation tolerance outcomes 6, suggesting potential clinical utility as a biomarker in transplant immunology.

Sources cited
1
FEM1C is a CRL2 substrate receptor recognizing Arg/C-degrons and specific target proteins
PMID: 33398168
2
Molecular mechanism of FEM1C C-degron recognition through semi-open binding pocket
PMID: 33398170
3
FEM1C mediates SLBP ubiquitination and degradation across cell cycle stages
PMID: 28118078
4
FEM1C is a conserved third member of the FEM1 gene family in vertebrates
PMID: 14527725
5
De novo FEM1C variants cause neurodevelopmental disorder with developmental delay, pyramidal signs and ataxia
PMID: 36336956
6
FEM1C gene expression is a biomarker with predictive capacity for liver transplantation tolerance
PMID: 30720688
Disease Associationsⓘ20
AtaxiaOpen Targets
0.37Weak
cerebellar ataxiaOpen Targets
0.37Weak
Neurodevelopmental disorderOpen Targets
0.30Weak
neurodegenerative diseaseOpen Targets
0.23Weak
Intellectual disabilityOpen Targets
0.18Weak
colorectal carcinomaOpen Targets
0.07Suggestive
atrial fibrillationOpen Targets
0.03Suggestive
alcohol drinkingOpen Targets
0.03Suggestive
hypertensionOpen Targets
0.03Suggestive
Premature ovarian insufficiencyOpen Targets
0.02Suggestive
cardioembolic strokeOpen Targets
0.02Suggestive
atrial flutterOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
colorectal cancerOpen Targets
0.01Suggestive
bacteriemiaOpen Targets
0.01Suggestive
adenomaOpen Targets
0.01Suggestive
ataxia telangiectasiaOpen Targets
0.01Suggestive
tuberculosisOpen Targets
0.01Suggestive
Abnormal sperm morphologyOpen Targets
0.01Suggestive
dentin dysplasiaOpen Targets
0.01Suggestive
Pathogenic Variants1
NM_020177.3(FEM1C):c.376G>C (p.Asp126His)Likely pathogenic
not provided|Neurodevelopmental disorder
★☆☆☆2024→ Residue 126
View on ClinVar ↗
Related Genes
KLHDC2Protein interaction97%CUL2Protein interaction94%ZER1Protein interaction94%GLMNProtein interaction94%KLHDC10Protein interaction94%PRAMEProtein interaction94%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
43%
Brain
37%
Lung
29%
Liver
18%
Ovary
15%
Gene Interaction Network
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FEM1CKLHDC2CUL2ZER1GLMNKLHDC10PRAME
PROTEIN STRUCTURE
Preparing viewer…
PDB6XKC · 2.03 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.58Moderately Constrained
pLIⓘ
0.65Intermediate
Observed/Expected LoF0.38 [0.25–0.58]
RankingsWhere FEM1C stands among ~20K protein-coding genes
  • #12,790of 20,598
    Most Researched26
  • #5,297of 5,498
    Most Pathogenic Variants1
  • #3,939of 17,882
    Most Constrained (LOEUF)0.58 · top quartile
Genes detectedFEM1C
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Tolerance Biomarkers in Liver Transplantation: Independent External Validation of the Predictive Strength of SENP6 and FEM1C Gene Expression.
PMID: 30720688
Transplantation · 2019
1.00
2
Disruption of FEM1C-W gene in zebra finch: evolutionary insights on avian ZW genes.
PMID: 19139913
Chromosoma · 2009
0.90
3
FEM1 proteins are ancient regulators of SLBP degradation.
PMID: 28118078
Cell Cycle · 2017
0.80
4
The Fem1c genes: conserved members of the Fem1 gene family in vertebrates.
PMID: 14527725
Gene · 2003
0.70
5
Molecular basis for ubiquitin ligase CRL2
PMID: 33398170
Nat Chem Biol · 2021
0.60