Enzyme or Coenzyme

Valosin Containing Protein

A highly-conserved AAA ATPase that functions in the biogenesis of the transitional ENDOPLASMIC RETICULUM and fragmentation and reassembly of the GOLGI APPARATUS during MITOSIS. It also functions in a complex with UFD1L and NPLOC4 proteins to export misfolded ubiquitinated proteins from the endoplasmic reticulum and outer mitochondrial membrane to the cytoplasm for degradation by the PROTEASOME and also plays a role in AUTOPHAGY of ubiquitinated proteins. It occurs in neuronal INCLUSION BODIES from patients with AMYOTROPHIC LATERAL SCLEROSIS and LEWY BODIES from PARKINSON DISEASE patients.