TFAM
GeneName
TFAM
Summary
TFAM, also known as transcription factor 6, mtTFA, or mtTF1, is a 29 kDa mitochondrial protein that plays a pivotal role in the regulation of mitochondrial DNA transcription and replication. It is primarily localised in the mitochondrial matrix and nucleoid, where it binds to mitochondrial DNA to facilitate the assembly of the mitochondrial respiratory chain complexes. TFAM also exhibits chromatin binding activity and is involved in the positive regulation of DNA-templated transcription, particularly at mitochondrial promoters. Additionally, it has roles in cellular responses to hypoxia and nutrient availability.
Importance
TFAM is relevant to: - Mitochondrial dysfunction and associated diseases, including neurodegenerative disorders and metabolic syndromes, due to its essential role in mitochondrial DNA maintenance and transcription. - Ageing processes, as alterations in TFAM levels can impact mitochondrial biogenesis and function over time. - Cancer biology, where changes in mitochondrial gene expression may influence tumour metabolism and growth. - Studies of cellular responses to environmental stressors, such as hypoxia, highlighting its role in adaptive mechanisms.
Top Products
For researchers investigating TFAM, we recommend two excellent primary antibodies that cater to various experimental needs. The first is the well-cited polyclonal antibody, Anti-mtTFA antibody - Mitochondrial Marker (ab131607), which has garnered 172 citations, reflecting its strong reputation in the field. This antibody is particularly effective for Western blotting (WB), making it a reliable choice for protein detection.In addition, we offer the recombinant antibody, Anti-mtTFA antibody [EPR12285] - Mitochondrial Marker (ab176558). This product has been validated in knockout models and is suitable for a broader range of applications, including WB, immunohistochemistry (IHC), immunocytochemistry (ICC), and immunoprecipitation (IP). With 30 citations, it is gaining traction among researchers. The recombinant nature of this antibody ensures batch-to-batch consistency, making it an excellent option for those requiring dependable results in their TFAM studies.
Abcam Product Citation Summary
The data indicates that TFAM is frequently studied in the context of mitochondrial function and biogenesis across various species, particularly in mouse models. The applications primarily involve Western blotting, highlighting its importance in understanding mitochondrial DNA and gene expression, especially in conditions like diabetes, sepsis, and after ischemia-reperfusion injury.
Abcam Product Citation Table
Domain
Binds DNA via its HMG boxes. When bound to the mitochondrial light strand promoter, bends DNA into a U-turn shape, each HMG box bending the DNA by 90 degrees.
Function
Binds to the mitochondrial light strand promoter and functions in mitochondrial transcription regulation (PubMed:29445193, PubMed:32183942). Component of the mitochondrial transcription initiation complex, composed at least of TFB2M, TFAM and POLRMT that is required for basal transcription of mitochondrial DNA (PubMed:29149603). In this complex, TFAM recruits POLRMT to a specific promoter whereas TFB2M induces structural changes in POLRMT to enable promoter opening and trapping of the DNA non-template strand (PubMed:20410300). Required for accurate and efficient promoter recognition by the mitochondrial RNA polymerase (PubMed:22037172). Promotes transcription initiation from the HSP1 and the light strand promoter by binding immediately upstream of transcriptional start sites (PubMed:22037172). Is able to unwind DNA (PubMed:22037172). Bends the mitochondrial light strand promoter DNA into a U-turn shape via its HMG boxes (PubMed:1737790). Required for maintenance of normal levels of mitochondrial DNA (PubMed:19304746, PubMed:22841477). May play a role in organizing and compacting mitochondrial DNA (PubMed:22037171).
Involvement in disease
Mitochondrial DNA depletion syndrome 15, hepatocerebral type
MTDPS15
An autosomal recessive mitochondrial disorder characterized by severe intrauterine growth restriction, neonatal-onset hypoglycemia and liver dysfunction, mitochondrial DNA depletion in liver and skeletal muscle, and abnormal mitochondrial morphology observed in skeletal muscle. Hepatic pathology includes cirrhosis, steatosis and cholestasis. Progression to liver failure and death is rapid with no evidence of neurological impairment or other organ involvement.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Phosphorylation by PKA within the HMG box 1 impairs DNA binding and promotes degradation by the AAA+ Lon protease.
Cellular localization
- Mitochondrion
- Mitochondrion matrix
- Mitochondrion nucleoid
Alternative names
TCF6, TCF6L2, TFAM, mtTFA, Mitochondrial transcription factor 1, Transcription factor 6, Transcription factor 6-like 2, MtTF1, TCF-6