Ribosome binding factor A, mitochondrial (RBFA)

Ribosome binding factor A, mitochondrial (RBFA), is a mitochondrial RNA-binding protein involved in the late steps of mitoribosome small subunit maturation. In human mitochondria, RBFA binds specifically and predominantly to the 12S rRNA, particularly at the 3′-terminal region at helices 44 and 45[1]. This association facilitates the dimethylation of two consecutive adenines, an essential modification for the proper maturation of mitochondrial rRNA and subsequent assembly of functional mitoribosomes. RBFA is necessary for the quality control of mitochondrial ribosome assembly, preventing the formation of dysfunctional ribosomes and supporting mitochondrial gene expression. While functionally analogous to bacterial RbfA involved in cold-shock adaptation and 30S ribosomal subunit maturation[2][3], human RBFA operates within a distinct molecular context. Loss of RBFA disrupts mitochondrial ribosome biogenesis, impairs cell growth, and may contribute to cellular phenotypes associated with mitochondrial dysfunction, although it is not currently linked to specific disease states[1]. No direct small molecule modulators or clinical drugs targeting RBFA are known, and it does not serve as a clinical biomarker or drug safety concern.

Other names

Putative ribosome-binding factor A, mitochondrialRBFAC18orf22FLJ21172HsT169Ribosome binding factor A (putative)