DNAJB6 is a DnaJ/Hsp40 co-chaperone protein that functions primarily as a suppressor of protein aggregation, particularly huntingtin and α-synuclein 1. The protein operates through isoform-specific mechanisms, where isoform B (but not isoform A) inhibits huntingtin aggregation 1. DNAJB6 is widely expressed across tissues with highest levels in the brain, where it localizes to both neurons (68-80%) and oligodendrocytes (41-53%) 2. The protein functions in protein quality control, intermediate filament organization, and Z-disc maintenance 3. Clinically, DNAJB6 mutations cause limb-girdle muscular dystrophy type 1E (LGMD1E), an autosomal dominant myopathy characterized by progressive proximal muscle weakness 4. DNAJB6-associated LGMD shows increased prevalence in some populations and manifests as a distal myopathy form, contributing to myofibrillar myopathy pathology through disrupted protein aggregation pathways 56. Beyond muscle disease, altered DNAJB6 expression is implicated in neurodegenerative conditions including Parkinson's disease and Multiple System Atrophy, suggesting therapeutic potential as a drug development target 2. Recent evidence indicates circDNAJB6 derived from human milk exosomes can promote DNAJB6 transcription and ameliorate bronchopulmonary dysplasia in experimental models 7.