TRIP11 encodes Golgi microtubule-associated protein 210 (GMAP-210), which serves as an essential membrane tether required for vesicle tethering to the Golgi apparatus and maintenance of Golgi structure and function 1. The protein is critical for efficient anterograde and retrograde trafficking in the early secretory pathway, functioning at both the ER-to-Golgi intermediate compartment and Golgi complex 1. TRIP11 also functions as a transcriptional coactivator, binding the thyroid hormone receptor beta (THRB) in the presence of triiodothyronine and enhancing THRB-mediated transcription through a PI3K-dependent mechanism 2. The protein physically interacts with intraflagellar transport 20 (IFT20), linking Golgi function to ciliary trafficking and ciliogenesis 13. Loss-of-function mutations in TRIP11 cause achondrogenesis type 1A (ACG1A), a lethal skeletal dysplasia characterized by complete disruption of Golgi function, impaired extracellular matrix proteoglycan secretion, and defective chondrocyte maturation 13. Hypomorphic mutations result in the milder odontochondrodysplasia (ODCD), where residual GMAP-210 function maintains partial Golgi integrity but still produces skeletal abnormalities and dentinogenesis imperfecta 14. These disorders demonstrate a genotype-phenotype correlation based on the severity of TRIP11 dysfunction 4.