Target intelligence / Profile preview

ATPase family AAA domain-containing protein 1 (ATAD1)

Target
ATAD1
Molecular classification
Enzyme, AAA+ ATPase (ATPases Associated with diverse cellular Activities), Mitochondrial protein quality control factor
01

Overview

ATPase family AAA domain-containing protein 1 (ATAD1; also known as Thorase and Msp1) is a highly conserved AAA+ ATPase predominantly localized to the outer membrane of mitochondria and peroxisomes[1][3][4]. It plays a crucial role in cellular homeostasis and mitochondrial function by recognizing and extracting mislocalized tail-anchored membrane proteins, facilitating their degradation or correct retargeting and thereby preserving mitochondrial integrity[1][2][3][4]. In neurons, ATAD1 also regulates synaptic plasticity by disassembling postsynaptic protein complexes and controlling the internalization of AMPA receptors, which is critical for processes such as learning and memory[1][2][4]. ATAD1 is considered a potential therapeutic target in diseases associated with mitochondrial dysfunction and neurological disorders, as well as in cancer, reflecting its multifaceted roles in protein quality control and cellular signaling[1][4].

Other names
ThoraseMsp1Outer mitochondrial transmembrane helix translocaseFNP001hATAD1FLJ14600AFDC1HKPX4ATPase family AAA domain containing 1
02

Biological functions

Extraction of mislocalized proteins from outer mitochondrial membraneProtein quality controlMaintenance of mitochondrial proteostasisExtraction of stuck import substratesRegulation of synaptic plasticity (by mediating internalization of neurotransmitter receptors such as AMPA receptors)
03

Disease associations

Neurodegenerative diseaseCancerDisorders of mitochondrial dysfunction (e.g., hyperekplexia 4)
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Safety considerations

Loss or dysfunction of ATAD1 may lead to mitochondrial protein mislocalization, proteostasis defects, or synaptic dysfunction[1][2][3][4].Variants linked to neurological disorders (e.g., hyperekplexia 4)[1].

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