Multisystem proteinopathy

Multisystem proteinopathy (MSP) is a rare, inherited degenerative disease characterized by progressive dysfunction across multiple organ systems, primarily affecting muscle, bone, and the nervous system. Multisystem proteinopathy is an adult-onset, genetically heterogenous disease An individual with MSP typically develops one or more of these more common diseases: amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), inclusion body myopathy (IBM), Paget's disease of bone (PDB). The most common cause of MSP is a missense mutation affecting the valosin-containing protein (VCP) gene, but at least four other genes have been identified.