Cellular distribution and subcellular localization of spatacsin and spastizin, two proteins involved in hereditary spastic paraplegia

RP Murmu, E Martin, A Rastetter, T Esteves… - Molecular and Cellular …, 2011 - Elsevier
Truncating mutations in the SPG11 and SPG15 genes cause complicated spastic
paraplegia, severe neurological conditions due to loss of the functions of spatacsin and
spastizin, respectively. We developed specific polyclonal anti-spatacsin (SPG11) and anti-
spastizin (SPG15) antisera, which we then used to explore the intracellular and tissue
localizations of these proteins. We observed expression of both proteins in human and rat
central nervous system, which was particularly strong in cortical and spinal motor neurons …