A bacterial artificial chromosome (BAC) (#RP23-312I9) containing the entire mouse Lrrk2 (leucine-rich repeat kinase 2) gene was modified by targeted mutation of the Lrrk2 locus to harbor the Lrrk2-G2019S mutation associated with autosomal dominant, late-onset Parkinson's disease originally identified in Spanish families originating from the Basque region. This modified 240 kb BAC contains a FLAG-tag downstream from the start codon. Line 2 was established with expression in cerebral cortex, striatum, substantia nigra, internal capsule, and hippocampus at an approximately 6-8 fold greater level than endogenous mouse Lrrk2.