biostudies-literatureBerezniuk INIDDK NIH HHSNIDA NIH HHSFinanciadora de Estudos e ProjetosNational Institutes of Health1813-23https://www.ebi.ac.uk/biostudies/studies/S-EPMC2874472biostudies-literatureUnknown24(6)Purkinje cell degeneration (pcd) mice have a mutation within the gene encoding cytosolic carboxypeptidase 1 (CCP1/Nna1), which has homology to metallocarboxypeptidases. To assess the function of CCP1/Nna1, quantitative proteomics and peptidomics approaches were used to compare proteins and peptides in mutant and wild-type mice. Hundreds of peptides derived from cytosolic and mitochondrial proteins are greatly elevated in pcd mouse hypothalamus, amygdala, cortex, prefrontal cortex, and striatum. However, the major proteins detected on 2-D gel electrophoresis were present in mutant and wild-type mouse cortex and hypothalamus at comparable levels, and proteasome activity is normal in these brain regions of pcd mice, suggesting that the increase in cellular peptide levels in the pcd mice is duFASEB journal : official publication of the Federation of American Societies for Experimental BiologyCCP1/Nna1 functions in protein turnover in mouse brain: Implications for cell death in Purkinje cell degeneration mice.PMC2874472DA-04494A‐03/134DK‐51271DK-51271R01 DK051271R01 DA004494DA‐04494Sironi JBerezniuk IHirata IYCastro LMFerro ESFricker LDCallaway MBfalseCCP1/Nna1 functions in protein turnover in mouse brain: Implications for cell death in Purkinje cell degeneration mice.Purkinje cell degeneration (pcd) mice have a mutation within the gene encoding cytosolic carboxypeptidase 1 (CCP1/Nna1), which has homology to metallocarboxypeptidases. To assess the function of CCP1/Nna1, quantitative proteomics and peptidomics approaches were used to compare proteins and peptides in mutant and wild-type mice. Hundreds of peptides derived from cytosolic and mitochondrial proteins are greatly elevated in pcd mouse hypothalamus, amygdala, cortex, prefrontal cortex, and striatum. However, the major proteins detected on 2-D gel electrophoresis were present in mutant and wild-type mouse cortex and hypothalamus at comparable levels, and proteasome activity is normal in these brain regions of pcd mice, suggesting that the increase in cellular peptide levels in the pcd mice is du2010-01-01T00:00:00Z2010 Jun2025-04-26T06:16:32.36Z2019-03-27T00:30:57ZS-EPMC28744722006153510.1096/fj.09-147942