Members of the MTERF (mitochondrial transcription termination factor) family, including MTERF, MTERFD1, MTERFD2 and MTERFD3, are mitochondrial proteins that are that thought to be transcription termination factors. MTERF (mitochondrial transcription termination factor 1) is composed of three leucine zippers that form a three-stranded coiled-coil that binds to DNA. It is suggested that only the phosphorylated form of MTERF has transcription termination activity. MTERFD1 (MTERF domain containing 1) may play a role as a mitochondrial transcription regulator and is expressed as two isoforms produced by alternative splicing. MTERFD2 is a 381 amino acid protein that is encoded by a gene located on human chromosome 2q37.3. MTERFD3 is believed to be involved in cell cycle regulation and cell growth by modulating mitochondrial transcription. MTERFD3 is expressed in heart, skeletal muscle, pancreas and liver.
Function:
Regulator of mitochondrial ribosome biogenesis and translation. Binds to mitochondrial ribosomal RNAs 16S, 12S and 7S and targets NSUN4 RNA methyltransferase to the mitochondrial large ribosomal subunit (39S).
Subunit:
Heterodimer with NSUN4; this interaction may be required for NSUN4 recruitment to the mitochondrial large ribosomal subunit.
Subcellular Location:
Mitochondrion
Similarity:
Belongs to the mTERF family.
SWISS:
Q7Z6M4
Gene ID:
130916
Database links:
Entrez Gene: 130916 Human
Entrez Gene: 69821 Mouse
Entrez Gene: 363289 Rat
SwissProt: Q7Z6M4 Human
SwissProt: Q8BVN4 Mouse
SwissProt: Q4G078 Rat
Unigene: 159556 Human
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