RIMKLB (ribosomal modification protein rimK-like family member B), also known as NAAGS (N-acetyl-aspartyl-glutamate synthetase B), beta-citryl-glutamate synthase B or FAM80B, is a 386 amino acid cytoplasmic protein that belongs to the rimK family. Acting as the catalyst in the synthesis of beta-citryl-glutamate and N-acetyl-aspartyl-glutamate, RIMKLB contains one ATP-grasp domain and exists as two alternatively spliced isoforms. The gene encoding RIMKLB maps to human chromosome 12, which encodes over 1,100 genes and comprises approximately 4.5% of the human genome. Chromosome 12 is associated with a variety of diseases and afflictions, including hypochondrogenesis, achondrogenesis, Kniest dysplasia, Noonan syndrome and trisomy 12p, which causes facial developmental defects and seizure disorders.
