ATPase family AAA domain-containing protein 3A (ATAD3A)

ATPase family AAA domain-containing protein 3A (ATAD3A) is an integral mitochondrial inner membrane protein belonging to the AAA+ ATPase superfamily.[1][2][5] It contains an N-terminal coiled-coil domain, two transmembrane domains, and a C-terminal ATPase domain.[2][5] ATAD3A regulates mitochondrial nucleoid architecture by interacting with mtDNA and nucleoid-associated proteins, controls cholesterol transport within mitochondria-associated membranes (MAMs), and maintains cristae structure essential for mitochondrial energy production.[1][2][3][8] It participates in multiple cellular processes including cell proliferation pathways and is required for proper mitochondrial function and membrane organization.[2][7][8] Mutations or dysfunction of ATAD3A are causative in severe neurological disorders (including some mitochondrial diseases and neurodegenerative syndromes) and have been implicated in cancer and cholesterol regulation.[1][2][3][6][8] ATAD3A is increasingly recognized as a potential therapeutic target, though direct drug modulators are primarily experimental (peptide inhibitors). Safety concerns are significant due to its fundamental roles in cellular metabolism and structure[3][6].

Other names

ATAD3AFLJ10709HAYOSPHRINLATPase family AAA domain-containing protein 3A

Peptide inhibitors (e.g., DA1) block ATAD3A oligomerization, reducing mitochondrial fragmentation and neurodegeneration