Cytosolic carboxypeptidase 5 (AGBL5)

Cytosolic carboxypeptidase 5 (AGBL5) is a metallocarboxypeptidase enzyme responsible for the deglutamylation of proteins, primarily acting on α- and β-tubulin by cleaving glutamate residues from polyglutamate side chains or carboxyl termini[1][3][5]. This activity modulates microtubule dynamics, which is critical for cellular processes in specialized cells including retinal photoreceptors and germ cells. AGBL5 has a dual substrate specificity, removing both α- and γ-linked glutamate residues. Mutations in AGBL5 are a genetically confirmed cause of autosomal recessive retinitis pigmentosa, implicating its essential role in maintaining retinal structure and function[1][4]. Animal models indicate involvement in spermatogenesis, where loss of function leads to impaired microtubule organization and male infertility[1]. AGBL5 may also contribute to antiviral immunity by the deglutamylation of cGAS[3]. Currently, no drugs are known to directly target this enzyme, and its primary biomedical relevance is as a genetic biomarker in inherited retinal diseases.

Other names

CCP5RP75ATP/GTP-binding protein-like 5protein deglutamylase CCP5cytosolic carboxypeptidase-like protein 5CBPC5_HUMANFLJ21839