Anti-PTCD3 / Pentatricopeptide repeat domain-containing protein 3

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. PTCD3 antibody detects Pentatricopeptide repeat domain-containing protein 3, encoded by the PTCD3 gene on chromosome 2p11.2. PTCD3 antibody is used to study this mitochondrial RNA-binding protein required for mitochondrial translation and respiratory function. PTCD3 belongs to the large pentatricopeptide repeat (PPR) protein family, which regulates RNA metabolism in organelles. It is localized to the mitochondrial matrix, where it participates in ribosome assembly and translation initiation for mitochondrial-encoded genes. Expression is ubiquitous but highest in tissues with high metabolic demand such as heart, brain, and muscle.Structurally, PTCD3 contains multiple PPR motifs that form helix-turn-helix repeats mediating RNA binding. These motifs allow PTCD3 to recognize specific RNA sequences, stabilizing ribosome-mRNA complexes and supporting efficient translation. PTCD3 associates with the small subunit of the mitochondrial ribosome (28S subunit), acting as a structural and functional component of the translation machinery.Functionally, PTCD3 ensures proper mitochondrial protein synthesis by stabilizing ribosomal complexes and supporting initiation of translation. Knockdown or mutation disrupts translation of mitochondrial mRNAs, leading to impaired oxidative phosphorylation complex assembly and respiratory chain dysfunction. PTCD3 is also thought to regulate specific RNA processing events, although its exact targets remain under investigation. Researchers use PTCD3 antibody to investigate mitochondrial translation, ribosome biology, and energy metabolism.Clinically, mutations in PTCD3 have been associated with mitochondrial encephalopathy, a disorder characterized by neurological dysfunction, muscle weakness, and lactic acidosis. PTCD3 deficiency results in combined oxidative phosphorylation defects, underscoring its essential role in mitochondrial function. Beyond inherited disorders, PTCD3 expression changes have been reported in cancers, reflecting the importance of mitochondrial activity in tumor metabolism. NSJ Bioreagents offers PTCD3 antibody for studies in mitochondrial biology, disease pathogenesis, and metabolic regulation.Experimentally, PTCD3 antibody is applied in western blotting to detect the ~79 kDa protein, in immunofluorescence microscopy to confirm mitochondrial localization, and in immunohistochemistry to study tissue-specific expression. Immunoprecipitation with PTCD3 antibody allows isolation of mitochondrial ribosome complexes for functional analysis.