TRIM13: Tripartite Motif Containing 13

A RING-type E3 ubiquitin ligase involved in endoplasmic reticulum-associated degradation and tumor suppression.

Gene Information Card

Symbol TRIM13
Full Name Tripartite Motif Containing 13
Gene Type Protein coding
Chromosomal Location 13q14.2
NCBI Gene ID 10206 ncbi.nlm.nih.gov/gene/10206
Ensembl ID ENSG00000139618
UniProt ID O60858
OMIM ID 606760
HGNC ID 9977
Aliases RFP2, LEU5, RNF77

Description

TRIM13 (Tripartite Motif Containing 13) encodes a member of the tripartite motif (TRIM) family. The protein contains a RING-type zinc finger, a B-box, and a coiled-coil domain, and functions as an E3 ubiquitin ligase. It localizes to the endoplasmic reticulum (ER) membrane and is involved in ER-associated degradation (ERAD), ubiquitination, and regulation of NF-kappaB signaling. TRIM13 is considered a tumor suppressor gene, frequently deleted or downregulated in B-cell chronic lymphocytic leukemia and multiple myeloma.

Disease Associations

Disease category Pathophysiological mechanism Genomic evidence
B-cell chronic lymphocytic leukemia Deletion or epigenetic silencing of TRIM13 on 13q14 contributes to leukemogenesis PMID: 12460929; COSMIC
Multiple myeloma Loss of TRIM13 expression associated with disease progression and poor prognosis PMID: 17690171; COSMIC
Breast cancer Reduced TRIM13 expression correlates with tumor aggressiveness PMID: 25329317

Expression Profile

Tissue Expression
Tissue nTPM level
Bone marrow 8.2 Medium
Lymph node 6.5 Medium
Spleen 5.1 Low
Testis 4.3 Low
Brain 2.1 Not detected
Cell Line Expression
Cell Line nTPM Notes
K-562 (leukemia) 12.4 High expression
HEK 293 (embryonic kidney) 7.8 Moderate expression
HeLa (cervical cancer) 5.2 Low expression
MCF7 (breast cancer) 3.1 Very low expression
Data source:Human Protein Atlas(proteinatlas.org)

Mutations & Variants

Hotspot Mutations
Variant Type Frequency Functional Description
c.1A>G (p.Met1?) Missense 0.01% (gnomAD) Loss of start codon; predicted loss of function
c.337C>T (p.Arg113*) Nonsense 0.002% (COSMIC) Premature truncation; loss of E3 ligase activity
c.494_495del (p.Glu165Glyfs*12) Frameshift 0.001% (COSMIC) Frameshift; loss of function
c.788G>A (p.Arg263Gln) Missense 0.005% (ClinVar) Uncertain significance; possibly damaging
Mutation functional classification

Loss of Function (LOF)

Nonsense and frameshift mutations (e.g., p.Arg113*, p.Glu165Glyfs*12) abolish E3 ubiquitin ligase activity, impairing ERAD and tumor suppression.

Gain of Function (GOF)

No gain-of-function mutations have been reported for TRIM13.

Dominant Negative (DN)

No dominant-negative mutations have been characterized for TRIM13.

Pathways

• Endoplasmic reticulum-associated degradation (ERAD) pathway
• Ubiquitin-proteasome pathway
• NF-kappaB signaling (negative regulation)

Protein Summary

TRIM13 is a 403-amino acid protein (UniProt O60858) with a molecular weight of ~46 kDa. It contains an N-terminal RING finger domain (residues 24-67) responsible for E3 ubiquitin ligase activity, a B-box domain (residues 95-138), and a coiled-coil region (residues 145-200). The protein is anchored to the ER membrane via a C-terminal transmembrane domain (residues 370-390). TRIM13 mediates ubiquitination of misfolded ER proteins and targets them for proteasomal degradation. It also negatively regulates NF-kappaB activation by promoting ubiquitination of IKKgamma/NEMO.

Related Products

Product name Cat.No. Species Gene ID
TRIM13 Knockout HEK293 Cell Line EDJ-KQ6949 Human 10206 Details Get a Quote
TRIM13 Knockout A-549 Cell Line EDJ-KQ31613 Human 10206 Details Get a Quote
TRIM13 Knockout HCT 116 Cell Line EDJ-KQ31614 Human 10206 Details Get a Quote
TRIM13 Knockout HeLa Cell Line EDJ-KQ31615 Human 10206 Details Get a Quote
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