PLP-binding protein

PLP-binding protein is a protein that in humans is encoded by the PLPBP gene (previously PROSC). This protein binds to pyridoxal phosphate (PLP), the active form of vitamin B6, and is involved in regulating its levels inside of the cell.

Clinical significance Mutations of the PROSC gene cause early-onset vitamin B6-dependent epilepsy (EPVB6D), a disease first described in 2016.

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