Target intelligence / Profile preview

AFG1-like ATPase (AFG1L)

Target
AFG1L
Molecular classification
Enzyme, AAA+ ATPase, Mitochondrial integral membrane protein, Protein quality control factor
01

Overview

AFG1-like ATPase (AFG1L) is a mitochondrial AAA+ ATPase and integral membrane protein involved in protein quality control within the mitochondrial matrix and at the inner mitochondrial membrane[3][4][5][1]. It is crucial for the degradation of misfolded or excess nuclear-encoded respiratory complex IV (cytochrome c oxidase) subunits and for maintaining mitochondrial morphology and proteostasis[3][5][4]. Its activity safeguards mitochondrial fidelity, supports oxidative stress tolerance, and preserves overall cellular and organismal health, as shown in yeast and metazoan models[1][5]. In humans, the protein is referred to as LACE1, and variants in the gene have been implicated in disorders such as bipolar disorder and marasmus[3]. There are no known direct pharmacological modulators or drugs that target AFG1-like ATPase.

Other names
AFG1-like ATPaseAFG1LAFG1LACE1Lactation elevated protein 1Protein AFG1 homologATPase family gene 1 homolog (S. cerevisiae)c222389CG8520 gene productlactation elevated protein 1
02

Mechanism of action

Not applicable. No drugs directly target this protein as of the current literature. Its mechanisms involve ATP hydrolysis–dependent unfolding and degradation of misfolded mitochondrial proteins, and aiding turnover/extraction of inner mitochondrial membrane protein subunits[1][5].

03

Biological functions

Mitochondrial protein homeostasisProtein quality control (proteostasis) in the mitochondrial matrixDegradation of mitochondrial and nuclear-encoded complex IV subunitsMitochondrial morphology maintenanceTolerance to oxidative stressRegulation of respiratory chain complexes
04

Disease associations

Neurodegenerative disease (suggested by mitochondrial function and protein homeostasis roles)Bipolar disorder (gene polymorphism association)[3]Marasmus (gene association)[3]Reduced lifespan and mitochondrial dysfunction in model organisms[1]
05

Safety considerations

None specifically reported; loss-of-function may compromise mitochondrial integrity and cell survival, leading to neurodegeneration or reduced lifespan in model organisms[1]

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