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ATAD3A and ATAD3B (MIM 612317) are mitochondrial membrane proteins that contribute to the stabilization of large mitochondrial DNA (mtDNA)-protein complexes called nucleoids (He et al., 2007 [PubMed 17210950]).[supplied by OMIM, Sep 2008].. Additionally we are shipping ATAD3A Antibodies (47) and ATAD3A Proteins (6) and many more products for this protein.
ATAD3A region deletions cause cerebellar dysfunction associated with altered mitochondrial DNA and cholesterol metabolism.
identification of a recurrent de novo ATAD3A c.1582C>T (p.Arg528Trp) variant by whole-exome sequencing in five unrelated individuals with a core phenotype of global developmental delay, hypotonia, optic atrophy, axonal neuropathy, and hypertrophic cardiomyopathy
Mutations in ATAD3A can thus be dominantly inherited and underlie variable neurological phenotypes, including hereditary spastic paraplegia with intrafamiliar variability. This finding extends the group of mitochondrial inner membrane AAA (show APP ELISA Kits) proteins associated with spasticity.
stabilization of WASF3 (show WASF3 ELISA Kits) function occurs through its interaction with ATAD3A and GRP78 (show HSPA5 ELISA Kits), which may provide a bridge between the ER and mitochondria, allowing communication between the two organelles
These results demonstrate that ATAD3-mediated mitochondrial membrane formation participates in the optimal transfer of cholesterol from the endoplasmic reticulum into mitochondria.
High ATAD3A expression correlates with radioresistance in glioblastoma.
exploring ATAD3 mRNA expression, we confirmed the existence of an alternative splicing in rodent and of several mRNA isoforms in human
ATAD3B (show ATAD3B ELISA Kits) is a negative regulator of ATAD3A and may function as an adaptor of mitochondrial homeostasis and metabolism in human pluripotent embryonic stem cells and cancer cells.
Engineered a high copy strain expressing human ATAD3A-Myc (show MYC ELISA Kits)-HIS at a relative high level (2.5mg/l of yeast culture)without significantly affecting yeast growth.Urea-denaturated ATAD3A-Myc (show MYC ELISA Kits)-HIS bound to agarose-nickel beads and could be renatured and eluted.
ATAD3 may be implicated in an unknown but essential and growth-linked mitochondrial function existing since pluri-cellular organization and involved in tumorigenesis.
ATAD3 is present in five native mitochondrial protein (show COX6B2 ELISA Kits) complexes of different sizes, indicating complex roles of the protein in mitochondrial architecture and function.
ATAD3A and ATAD3B (MIM 612317) are mitochondrial membrane proteins that contribute to the stabilization of large mitochondrial DNA (mtDNA)-protein complexes called nucleoids (He et al., 2007
ATPase family, AAA domain containing 3A
, ATPase family AAA domain-containing protein 3A
, AAA-ATPase TOB3
, ATPase family AAA domain-containing protein 3
, ATPase family, AAA domain containing 3
, ATPase family, AAA domain containing 3B