LRRC7 (leucine rich repeat containing 7) encodes Densin-180, a brain-specific postsynaptic scaffold protein essential for synaptic function and neurodevelopment 1. The protein belongs to the leucine rich repeat and PDZ domain (LAP) protein family and is selectively expressed in neurons, functioning as a postsynaptic scaffold at glutamatergic synapses 1. Mechanistically, Densin-180 links cytoskeletal elements with signaling proteins such as the α-subunit of Ca2+/calmodulin-dependent protein kinase II and interacts with protein phosphatase 1 (PP1) through its LRR domain 1. The protein plays crucial roles in synaptic targeting, with PDZ domain variants interfering with proper synaptic localization in cultured neurons 1. Disease-wise, heterozygous missense or loss-of-function variants in LRRC7 cause a dominant neurodevelopmental disorder affecting 33 identified individuals, characterized by intellectual disability, autism, ADHD, aggression, and hyperphagia-associated obesity 12. The gene has also been implicated in Alzheimer's disease through genome-wide association studies 3. Clinically, LRRC7 represents a major determinant of intellectual development and behavior, with variants reducing protein binding capacity and synaptic function 1. A splice variant exists that is expressed ubiquitously, while the brain-specific Densin-180 isoform is critical for neuronal function 4.