PEP-19 immunohistochemistry defines the basal ganglia and associated structures in the adult human brain, and is dramatically reduced in Huntington's disease

The Calmodulin Regulator Protein, PEP-19, Sensitizes ATP-induced Ca2+ Release

“…In addition, PEP-19 has anti-apoptotic activity when expressed in PC12 and HEK293T cells (9,10), and it provides protection against Ca 2ϩ overload in cortical neurons (10). These experimental observations are consistent with a proposed neuroprotective role for PEP-19 based on expression patterns in neuronal tissues that are susceptible to Huntington and Alzheimer diseases (11).…”

Protective Effect of DY-9760e, a Calmodulin Antagonist, against Neuronal Cell Death

“…Furthermore, with respect to calmodulin-mediated cell death, there are several intriguing reports: 1) PEP-19, a brain-specific protein and negative regulator of calmodulin, can inhibit apoptotic processes in PC12 cells following UV irradiation or staurosporine treatment 31) ; and 2) immunoreactivity for PEP-19 is dramatically reduced in the brains from patients with Alzheimer's and Huntington's disease. 32) These data indicate that calmodulin may also contribute to neuronal loss in neurodegenerative diseases.…”

PCP4 regulates Purkinje cell excitability and cardiac rhythmicity

“…Interestingly, PCP4 is reportedly downregulated in selected brain regions in a number of neurologic syndromes, including Parkinson disease (26), Huntington disease (27), and chronic alcoholism (28). Moreover, a genetic mouse model of Huntington disease phenocopies the autonomic dysregulation seen in the Pcp4-null animals (29).…”