ATAD1

Atad1 is an ATPase that plays a critical role in regulating the surface expression of AMPA receptors (AMPAR), thereby regulating synaptic plasticity and learning and memory. It is required for NMDA-stimulated AMPAR internalization and inhibition of GRIA1 and GRIA2 recycling back to the plasma membrane; these activities are ATPase-dependent.

Full Name

ATPase Family, AAA Domain Containing 1

Function

Outer mitochondrial translocase required to remove mislocalized tail-anchored transmembrane proteins on mitochondria (PubMed:24843043).
Specifically recognizes and binds tail-anchored transmembrane proteins: acts as a dislocase that mediates the ATP-dependent extraction of mistargeted tail-anchored transmembrane proteins from the mitochondrion outer membrane (By similarity).
Also plays a critical role in regulating the surface expression of AMPA receptors (AMPAR), thereby regulating synaptic plasticity and learning and memory (By similarity).
Required for NMDA-stimulated AMPAR internalization and inhibition of GRIA1 and GRIA2 recycling back to the plasma membrane; these activities are ATPase-dependent (By similarity).

Biological Process

Extraction of mislocalized protein from mitochondrial outer membrane Source: UniProtKB
Learning Source: UniProtKB
Memory Source: UniProtKB
Negative regulation of synaptic transmission, glutamatergic Source: UniProtKB
Positive regulation of receptor internalization Source: UniProtKB
Protein import into peroxisome membrane Source: Reactome
Regulation of postsynaptic neurotransmitter receptor internalization Source: Ensembl

Cellular Location

Mitochondrion outer membrane; Postsynaptic cell membrane; Peroxisome membrane

Involvement in disease

Hyperekplexia 4 (HKPX4): An autosomal recessive severe neurologic disorder apparent from birth. HKPX4 is characterized by little if any development, hypertonia, early-onset refractory seizures in some patients, and respiratory failure resulting in early death, mostly in the first months of life.

Topology

Mitochondrial intermembrane: 1-15 aa
Helical: 16-32 aa
Cytoplasmic: 33-361 aa