Target intelligence / Profile preview

ATPase family AAA domain-containing protein 3B (ATAD3B)

Target
ATAD3B
Molecular classification
AAA+ ATPase, Mitochondrial membrane protein, Protein remodeling chaperone family (AAA+ protein family, Classic Clade)[1][3], Other
01

Overview

ATPase family AAA domain-containing protein 3B (ATAD3B) is a mitochondrially localized AAA+ ATPase protein anchored in the inner mitochondrial membrane, where it forms complexes with ATAD3A, modulating mitochondrial network organization and homeostasis[1][2][3]. ATAD3B is predominantly expressed in human embryonic stem cells and is re-expressed in several cancers, where it acts as a dominant negative regulator of ATAD3A, interfering with its interaction with mitochondrial nucleoid complexes and contributing to mitochondrial fragmentation[2][3]. ATAD3B belongs to a subfamily of chaperone-like AAA+ ATPases structurally related to protein remodeling factors, and has been connected to cell proliferation, stem cell pluripotency, and cancer progression[1][4]. Defects or dysregulated expression of ATAD3B are implicated in mitochondrial diseases and various malignancies, although no specific drugs or targeted therapies are currently approved for ATAD3B modulation[3]. The protein's compartmentalized structure and unique dual localization (contacting both intermembrane space and potentially cytosol) suggest roles in mitochondrial-cytosolic communication and the regulation of metabolic and genetic homeostasis within the organelle[1].

Other names
ATPase family AAA domain-containing 3BKIAA1273TOB3AAA-TOB3ATAD3BAAA-ATPase TOB3ATPase family AAA domain containing 3B
02

Mechanism of action

Not applicable (no drugs currently target ATAD3B specifically)[3]

03

Biological functions

Mitochondrial network organization[3]Regulation of mitochondrial homeostasis and metabolism[2][3]Negative regulator of ATAD3A interaction with nucleoid complexes (involved in mtDNA organization)[2][3]Mitochondrial dynamics (fragmentation, cristae structure maintenance)[1][2]Possibly involved in protein quality control and communication between mitochondria and cytosol[1]Cell proliferation (stem cell pluripotency and cancer)[2][4]
04

Disease associations

Cancer (notably related to stemness, tumorigenesis, and prognosis in several cancers)[2][3][4]Mitochondrial disease[3]Central nervous system lipid disorders[4]Chromosome 1p36.33 duplication syndrome[3]Other
05

Safety considerations

Disruption may lead to pleiotropic mitochondrial defects (e.g., impaired metabolism, loss of cristae structure, defects in mtDNA organization, lipid metabolism disturbances)[1][2][3]Loss of mitochondrial homeostasis, with potential systemic effects
06

Interacting drugs

None identified in current literature and reference databases[3]

1 more in the full profile.

07

Biomarkers

Potential biomarker for pluripotent embryonic stem cells and cancer (expression is specific to these contexts)[2][3]Prognostic biomarker in select cancers (e.g., head and neck, lung, uterine cervical, prostate, glioma, and hepatocellular carcinoma)[4]

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