Target intelligence / Profile preview

Pentatricopeptide repeat-containing protein 2, mitochondrial (PTCD2)

Target
PTCD2
Molecular classification
RNA-binding protein, Pentatricopeptide repeat (PPR) protein family, Mitochondrial protein, Other
01

Overview

Pentatricopeptide repeat-containing protein 2, mitochondrial (PTCD2) is a mitochondrial RNA-binding protein belonging to the pentatricopeptide repeat (PPR) protein family, characterized by multiple tandem PPR motifs which mediate interactions with RNA[1][4][5][6][7]. It is implicated in the regulation of mitochondrial gene expression, particularly through its involvement in the translational regulation of the mitochondrially-encoded cytochrome c oxidase subunit III (COIII) mRNA; loss of PTCD2 results in a substantial reduction of COIII translation, decreased assembly and activity of mitochondrial respiratory chain complex IV (cytochrome c oxidase), and impaired mitochondrial respiration[1]. PTCD2 is also thought to interact with the mitochondrial ribosome, further implicating it in RNA processing and translation control within mitochondria[1][6][7]. Disruption of PTCD2 has been associated with mitochondrial diseases such as mitochondrial DNA depletion syndrome and mitochondrial complex IV deficiency[5]. PTCD2 is not currently considered a primary therapeutic drug target, and no interacting drugs or clinical biomarker applications are reported as of now[5][6][7].

Other names
PTCD2Pentatricopeptide repeat domain 2FLJ12598CTC-365E16.1pentatricopeptide repeat-containing protein 2, mitochondrial
02

Biological functions

Regulation of mitochondrial mRNA translation (notably COIII mRNA)Mitochondrial RNA processing/maturationRegulation of mitochondrial respiratory chain functionMitochondrion organizationRegulation of mRNA processing
03

Disease associations

Mitochondrial DNA depletion syndrome 4BMitochondrial complex IV deficiency, nuclear type 1Other

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