TRIM63
GeneName
TRIM63
Summary
TRIM63, also known as MuRF1 or IRF, is a 40 kDa E3 ubiquitin ligase that plays a vital role in muscle physiology and pathology. It is primarily expressed in skeletal and cardiac muscle tissues, where it localises to various structures including the cytoplasm, Z discs, and M bands of myofibrils. TRIM63 is involved in the regulation of muscle contraction and is implicated in the process of muscle atrophy, particularly during conditions such as disuse or disease. Its function is mediated through its ability to bind to titin and other proteins, facilitating protein ubiquitination and degradation. Additionally, TRIM63 has roles in the innate immune response and in the regulation of cardiac muscle hypertrophy.
Importance
TRIM63 is relevant to: - Understanding muscle wasting conditions, such as cachexia and sarcopenia, due to its role in muscle atrophy - Cardiac health, as it negatively regulates cardiac muscle hypertrophy, providing insights into heart disease mechanisms - The innate immune response, linking muscle biology with immune system function - The response to glucocorticoids and interleukin-1, which are important in inflammation and stress responses
Top Products
For researchers investigating TRIM63, we recommend the well-cited polyclonal antibody, Anti-MURF1 antibody (ab183094). This antibody has garnered 21 citations, highlighting its reliability and trust within the research community. It is particularly effective for Western blotting (WB) and immunohistochemistry (IHC), making it a valuable tool for various experimental setups. Its proven performance in these applications ensures that you can confidently explore the role of TRIM63 in your studies.
Abcam Product Citation Summary
The use of the Abcam antibody ab183094 for TRIM63 detection in soleus muscles of rats indicates a focus on muscle biology and potentially on muscle-related diseases or conditions. The study context suggests an interest in understanding the role of TRIM63 in muscle physiology.
Abcam Product Citation Table
Developmental stage
Expressed throughout all developmental stages.
Domain
The RING-type zinc finger mediates interaction with SUMO2 and localization to the nucleus. Also required for the E3 ubiquitin ligase activity (By similarity).
The B box-type zinc finger mediates homodimerization.
Function
E3 ubiquitin ligase. Mediates the ubiquitination and subsequent proteasomal degradation of CKM, GMEB1 and HIBADH. Regulates the proteasomal degradation of muscle proteins under amino acid starvation, where muscle protein is catabolized to provide other organs with amino acids. Inhibits de novo skeletal muscle protein synthesis under amino acid starvation. Regulates proteasomal degradation of cardiac troponin I/TNNI3 and probably of other sarcomeric-associated proteins. May play a role in striated muscle atrophy and hypertrophy by regulating an anti-hypertrophic PKC-mediated signaling pathway. May regulate the organization of myofibrils through TTN in muscle cells.
Involvement in disease
Cardiomyopathy, familial hypertrophic, 31
CMH31
A form of hypertrophic cardiomyopathy, a heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. CMH31 is an autosomal recessive form characterized by moderate to severe hypertrophy, high incidence of ventricular arrhythmias, extensive fibrosis, and frequent left ventricular dysfunction.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Protein modification; protein ubiquitination.
Tissue Specificity
Muscle specific. Selectively expressed in heart and skeletal muscle. Also expressed in the iris.
Cellular localization
- Cytoplasm
- Nucleus
- Cytoplasm
- Myofibril
- Sarcomere
- M line
- Cytoplasm
- Myofibril
- Sarcomere
- Z line
- Colocalizes with TNNI3 in myocytes (By similarity).
Alternative names
IRF, MURF1, RNF28, SMRZ, TRIM63, E3 ubiquitin-protein ligase TRIM63, Iris RING finger protein, Muscle-specific RING finger protein 1, RING finger protein 28, RING-type E3 ubiquitin transferase TRIM63, Striated muscle RING zinc finger protein, Tripartite motif-containing protein 63, MuRF-1, MuRF1