PANK2 encodes pantothenate kinase 2, a cytoplasmic enzyme catalyzing phosphorylation of pantothenate (vitamin B5) to 4'-phosphopantothenate, the first and rate-determining step of coenzyme A (CoA) biosynthesis 1. PANK2 is the only pantothenate kinase isoform with mitochondrial localization 1, positioning it to regulate cellular energy metabolism and lipid homeostasis. Mutations in PANK2 cause pantothenate kinase-associated neurodegeneration (PKAN), the most common form of neurodegeneration with brain iron accumulation (NBIA) 1. PKAN presents with progressive movement disorder, dystonia, dysarthria, cognitive decline, and retinitis pigmentosa, with characteristic "eye of the tiger" MRI sign in the globus pallidus reflecting iron accumulation 1. Pathogenic PANK2 mutations perturb neuronal CoA availability, disrupting metabolic balance and triggering neurodegeneration 2. Disease onset varies from early childhood (classic PKAN) to later with slower progression (atypical PKAN), occasionally with psychiatric symptoms 3. Currently only symptomatic treatment exists; deep brain stimulation improves dystonia, while pantethine supplementation and iron chelators show promise in preclinical and pilot studies 1. PANK2 mutations account for a subset of NBIA cases 4, highlighting CoA metabolism's critical role in neuronal iron homeostasis and axonal integrity.