Function
Binds to the mitochondrial light strand promoter and functions in mitochondrial transcription regulation. Component of the mitochondrial transcription initiation complex, composed at least of TFB2M, TFAM and POLRMT that is required for basal transcription of mitochondrial DNA. 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.
Biological Context
Subcellular Location: Mitochondrion; Mitochondrion matrix, mitochondrion nucleoid
Disease Association: 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. [The disease is caused by variants affecting the gene represented in this entry]
Product Specifications
Recombinant Human Transcription factor A, mitochondrial (TFAM) is a recombinant protein from Homo sapiens (Human), expressed in E.coli, covering amino acids 43-246aa, with N-terminal 6xHis-tagged tag, molecular weight 28.5kDa, purity Greater than 90% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications.
