TUBGCP4 (tubulin gamma complex component 4) is a structural component of the gamma-tubulin ring complex (γ-TuRC), which mediates microtubule nucleation at centrosomes 1. The γ-TuRC regulates minus-end nucleation of alpha-beta tubulin heterodimers that assemble into microtubule protafilaments, essential for centrosome duplication and spindle formation 1. Beyond its canonical cytoskeletal role, TUBGCP4 regulates autophagy homeostasis by competing with ATG3 to interact with ATG7, thereby inhibiting LC3B lipidation 1. Complete TUBGCP4 knockout causes embryonic lethality due to spindle assembly defects, while haploinsufficiency demonstrates dosage sensitivity 1. TUBGCP4 mutations cause microcephaly and chorioretinopathy (MCCRP3), an autosomal recessive neuro-ophthalmic disorder characterized by microcephaly, developmental delay, chorioretinopathy, and photoreceptor degeneration 23. Pathogenic variants reduce γ-TuRC levels, disrupting microtubule nucleation and organization, leading to abnormal nuclear morphology and aneuploidy 3. Zebrafish morpholino studies confirmed reduced head volume and chorioretinal dysplasia 3. While TUBGCP4 expression correlates with better prognosis in hepatocellular carcinoma, its primary clinical significance remains in developmental neurological and ophthalmologic disease.