Mitochondrial transcription factor A, abbreviated as ''TFAM'' or ''mtTFA'', is a
protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, res ...
that in humans is encoded by the ''TFAM''
gene
In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a b ...
.
Function
This gene encodes a
mitochondrial transcription factor
In molecular biology, a transcription factor (TF) (or sequence-specific DNA-binding factor) is a protein that controls the rate of transcription of genetic information from DNA to messenger RNA, by binding to a specific DNA sequence. The f ...
that is a key activator of mitochondrial transcription as well as a participant in mitochondrial genome replication. TFAM binds mitochondrial promoter DNA to aid transcription of the mitochondrial genome. Studies in mice have demonstrated that this gene product is required to regulate the mitochondrial genome copy number and is essential for
embryonic development. A mouse model for
Kearns–Sayre syndrome
Kearns–Sayre syndrome (KSS), oculocraniosomatic disorder or oculocranionsomatic neuromuscular disorder with ragged red fibers is a mitochondrial myopathy with a typical onset before 20 years of age. KSS is a more severe syndromic variant of chron ...
was produced when expression of this gene was eliminated by targeted disruption in heart and muscle cells.
TFAM is a double box
High-mobility group
High-Mobility Group or HMG is a group of chromosomal proteins that are involved in the regulation of DNA-dependent processes such as
transcription, replication, recombination, and DNA repair.
Families
The HMG proteins are subdivided into 3 supe ...
DNA-binding and bending protein. This bending action is important for mitochondrial transcription initiation in mammals, but not in yeasts with the homolog Abf2. TFAM may also participate in the packaging of the mitochondrial genome, as its binding activity is non-sequence-specific.
Interactions
TFAM has been shown to
interact
Advocates for Informed Choice, dba interACT or interACT Advocates for Intersex Youth, is a 501(c)(3) nonprofit organization using innovative strategies to advocate for the legal and human rights of children with intersex traits. The organizati ...
with
TFB1M
Dimethyladenosine transferase 1, mitochondrial; Transcription factor B1, mitochondrial is a mitochondrial enzyme that in is encoded by the ''TFB1M'' gene.
TFB1M is a mitochondrial methyltransferase, which uses S-adenosyl methionine to dimethylate ...
and
TFB2M
Dimethyladenosine transferase 2; transcription factor B2, mitochondrial is an enzyme that in humans is encoded by the ''TFB2M'' gene.
This protein is a transcription initiation factor for the mitochondrial RNA polymerase, POLRMT. Its paralog TFB1 ...
.
References
Further reading
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External links
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