Parkin, a ubiquitin ligase, is responsible for autosomal recessive juvenile parkinsonism (AR‐JP). We identified parkin‐associated endothelin receptor‐like receptor (Pael‐R) as a substrate of parkin, whose accumulation is thought to induce unfolded protein response (UPR) ‐mediated cell death, leading to dopaminergic neurodegeneration. To create an animal model of AR‐JP, we generated parkin knockout/Pael‐R transgenic (parkin‐ko/Pael‐R‐tg) mice. parkin‐ko/Pael‐R‐tg mice exhibited early and progressive loss of dopaminergic as well as noradrenergic neurons without formation of inclusion bodies, recapitulating the pathological features of AR‐JP. Evidence of activation of UPR and up‐regulation of dopamine and its metabolites were observed throughout the lifetime. Moreover, complex I activity of mitochondria isolated from parkin‐ko/Pael‐R‐tg mice was significantly reduced later in life. These findings suggest that persistent induction of unfolded protein stress underlies chronic progressive catecholaminergic neuronal death, and that dysfunction of mitochondrial complex I and oxidative stress might be involved in the progression of Parkinson’s disease. parkin‐ko/Pael‐R‐tg mice represents an AR‐JP mouse model displaying chronic and selective loss of catecholaminergic neurons.