Target intelligence / Profile preview

Ribosomal modification protein RimK-like family member B (RIMKLB)

Target
RIMKLB
Molecular classification
Enzyme (specifically, a ligase), Ribosomal modification protein (RimK-like family)
01

Overview

Ribosomal modification protein RimK-like family member B (**RIMKLB**) is a cytosolic enzyme that catalyzes the ATP-dependent formation of beta-citryl-L-glutamate and N-acetyl-L-aspartyl-L-glutamate, two metabolites implicated in central nervous system biology[3][4]. It belongs to the RimK protein family, originally described as glutamyl ligases that post-translationally modify ribosomal proteins in bacteria, but RIMKLB in humans primarily acts as a metabolic ligase. Its activity is central to certain amino acid metabolic pathways and may influence neurological health, as disease associations include Canavan disease and leukodystrophies[3]. There are currently no approved therapeutics known to modulate its activity, nor validated biomarkers or direct safety concerns linked to its targeting. The gene is widely known by many aliases, but RIMKLB is the canonical gene symbol[3][4].

Other names
Beta-citrylglutamate synthase BFAM80BKIAA1238NAAG synthetase BNAAGS (sometimes NAAGS-I)N-acetyl-aspartylglutamate synthetase BRibosomal protein S6 modification-like protein BFamily with sequence similarity 80, member B
02

Mechanism of action

Not defined; as an enzyme, potential drugs would likely inhibit or modulate its ligase activity.

03

Biological functions

Synthesis of beta-citryl-L-glutamateSynthesis of N-acetyl-L-aspartyl-L-glutamateGlutamine family amino acid metabolism
04

Disease associations

Associated with Canavan diseaseAssociated with leukodystrophy
05

Safety considerations

None reported; therapeutic challenges would likely relate to metabolic impact if modulated, especially in the CNS due to involvement in NAAG synthesis
06

Biomarkers

None specifically described for patient selection or efficacy monitoring; possible relevance in diseases involving altered beta-citryl-L-glutamate or NAAG metabolism

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