Deleterious genetic changes in AGTPBP1 result in teratozoospermia with sperm head and flagella defects. | Deleterious genetic changes in AGTPBP1 result in teratozoospermia with sperm head and flagella defects. Lin YH, Wang YY, Lai TH, Teng JL, Lin CW, Ke CC, Yu IS, Lee HL, Chan CC, Tung CH, Conrad DF, O'Bryan MK, Lin YH., Free PMC Article | 02/8/2024 |
CCP1, a Regulator of Tubulin Post-Translational Modifications, Potentially Plays an Essential Role in Cerebellar Development. | CCP1, a Regulator of Tubulin Post-Translational Modifications, Potentially Plays an Essential Role in Cerebellar Development. Pang B, Araki A, Zhou L, Takebayashi H, Harada T, Kadota K., Free PMC Article | 03/30/2023 |
Nna1, Essential for Purkinje Cell Survival, Is also Associated with Emotion and Memory. | Nna1, Essential for Purkinje Cell Survival, Is also Associated with Emotion and Memory. Zhou L, Konno K, Yamazaki M, Abe M, Natsume R, Watanabe M, Takebayashi H, Sakimura K., Free PMC Article | 01/28/2023 |
The AGTPBP1 gene in neurobiology. | The AGTPBP1 gene in neurobiology. Lalonde R, Strazielle C. | 12/11/2021 |
Nna1 gene deficiency triggers Purkinje neuron death by tubulin hyperglutamylation and ER dysfunction. | Nna1 gene deficiency triggers Purkinje neuron death by tubulin hyperglutamylation and ER dysfunction. Li J, Snyder EY, Tang FHF, Pasqualini R, Arap W, Sidman RL., Free PMC Article | 06/12/2021 |
we demonstrated that the Agtpbp1 gene mutation induces a p53-dependent nucleolar stress response in PCs, which is characterized by nucleolar fragmentation, nucleoplasmic and cytoplasmic mislocalization of nucleolin, and dysfunction of both pre-rRNA processing and mRNA translation. | Nucleolin reorganization and nucleolar stress in Purkinje cells of mutant PCD mice. Baltanás FC, Berciano MT, Tapia O, Narcis JO, Lafarga V, Díaz D, Weruaga E, Santos E, Lafarga M. | 01/11/2020 |
CCP1 promotes mitochondrial fusion and motility to prevent Purkinje cell neuron loss in Purkinje cell degeneration mice. | CCP1 promotes mitochondrial fusion and motility to prevent Purkinje cell neuron loss in pcd mice. Gilmore-Hall S, Kuo J, Ward JM, Zahra R, Morrison RS, Perkins G, La Spada AR., Free PMC Article | 10/26/2019 |
We report the generation of a functional Nna1 conditional allele and possible mechanisms of Purkinje cell death in the Nna1 knockout in the cerebellum | Deletion of exons encoding carboxypeptidase domain of Nna1 results in Purkinje cell degeneration (pcd) phenotype. Zhou L, Hossain MI, Yamazaki M, Abe M, Natsume R, Konno K, Kageyama S, Komatsu M, Watanabe M, Sakimura K, Takebayashi H. | 10/12/2019 |
Lack of Cytosolic Carboxypeptidase 1 Leads to Subfertility due to the Reduced Number of Antral Follicles in pcd3J-/- Females. | Lack of Cytosolic Carboxypeptidase 1 Leads to Subfertility due to the Reduced Number of Antral Follicles in pcd3J-/- Females. Song N, Kim N, Xiao R, Choi H, Chun HI, Kang MH, Kim JH, Seo K, Soundrarajan N, Do JT, Song H, Ge ZJ, Park C., Free PMC Article | 06/4/2016 |
Nna1 and CCP4 remove the C-terminal glutamate from substrates with two or more glutamates, whereas CCP6 requires four or more glutamates. CCP4 behaves as a promiscuous glutamase with little preference for chain length or neighboring amino acid composition | Comparison of the enzymatic and functional properties of three cytosolic carboxypeptidase family members. Wu HY, Rong Y, Correia K, Min J, Morgan JI., Free PMC Article | 04/25/2015 |
The lack of CCP1/Nna1 leads to decreases in cellular levels of amino acids, which leads to elevated autophagy as a protective response to cellular amino acid starvation. | A defect in cytosolic carboxypeptidase 1 (Nna1) causes autophagy in Purkinje cell degeneration mouse brain. Berezniuk I, Fricker LD., Free PMC Article | 08/31/2013 |
The nna1 deficiency demonistrated that gliosis occurring during mitral cells and Purkinje cells death in the PCD mouse. | Differential glial activation during the degeneration of Purkinje cells and mitral cells in the PCD mutant mice. Baltanás FC, Berciano MT, Valero J, Gómez C, Díaz D, Alonso JR, Lafarga M, Weruaga E. | 06/1/2013 |
This study characterized the structure, expression and activity of wild type and mutated Nna1 in Purkinje cell degeneration (pcd) mice. | A structural and functional analysis of Nna1 in Purkinje cell degeneration (pcd) mice. Wu HY, Wang T, Li L, Correia K, Morgan JI., Free PMC Article | 02/2/2013 |
These data indicated that serotonergic function in the MOB is closely related to olfactory activity and that mitral cell loss induces serotonergic plastic responses. | Changes in the serotonergic system and in brain-derived neurotrophic factor distribution in the main olfactory bulb of pcd mice before and after mitral cell loss. Gómez C, Curto GG, Baltanás FC, Valero J, O'Shea E, Colado MI, Díaz D, Weruaga E, Alonso JR. | 05/19/2012 |
These results demonstrate a role for CCP1 in the processing of Glu residues from beta- as well as alpha-tubulin in vitro and in vivo. | Cytosolic carboxypeptidase 1 is involved in processing α- and β-tubulin. Berezniuk I, Vu HT, Lyons PJ, Sironi JJ, Xiao H, Burd B, Setou M, Angeletti RH, Ikegami K, Fricker LD., Free PMC Article | 04/28/2012 |
Agtpbp1 plays an important role in spermatogenesis and is important for survival of germ cells at spermatocytes stage onward | Abnormal sperm development in pcd(3J)-/- mice: the importance of Agtpbp1 in spermatogenesis. Kim N, Xiao R, Choi H, Jo H, Kim JH, Uhm SJ, Park C., Free PMC Article | 09/24/2011 |
Study analyzes Purkinje cell degeneration (pcd) mice that lack functional CCP1 and shows that microtubule hyperglutamylation is directly linked to neurodegeneration. | A family of protein-deglutamylating enzymes associated with neurodegeneration. Rogowski K, van Dijk J, Magiera MM, Bosc C, Deloulme JC, Bosson A, Peris L, Gold ND, Lacroix B, Bosch Grau M, Bec N, Larroque C, Desagher S, Holzer M, Andrieux A, Moutin MJ, Janke C. | 12/11/2010 |
This study provided insight into Nna1's role in neuronal development and why its absence renders Purkinje cells more vulnerable. | Nna1 mediates Purkinje cell dendritic development via lysyl oxidase propeptide and NF-κB signaling. Li J, Gu X, Ma Y, Calicchio ML, Kong D, Teng YD, Yu L, Crain AM, Vartanian TK, Pasqualini R, Arap W, Libermann TA, Snyder EY, Sidman RL., Free PMC Article | 10/23/2010 |
This studies indicated that Nna1 loss of function results in altered bioenergetics and mitochondrial dysfunction. | Mitochondrial dysfunction in NnaD mutant flies and Purkinje cell degeneration mice reveals a role for Nna proteins in neuronal bioenergetics. Chakrabarti L, Zahra R, Jackson SM, Kazemi-Esfarjani P, Sopher BL, Mason AG, Toneff T, Ryu S, Shaffer S, Kansy JW, Eng J, Merrihew G, MacCoss MJ, Murphy A, Goodlett DR, Hook V, Bennett CL, Pallanck LJ, La Spada AR., Free PMC Article | 08/9/2010 |
loss of Nna1 in Purkinje cells leads directly to their degeneration and Nna1's carboxypeptidase domain is essential for survival of these neurons | The carboxypeptidase-like substrate-binding site in Nna1 is essential for the rescue of the Purkinje cell degeneration (pcd) phenotype. Wang T, Parris J, Li L, Morgan JI. | 01/21/2010 |
identifed Nna1 as the gene mutated in the original Purkinje cell degeneration(pcd)and two additional pcd alleles (pcd2J and pcd3J) | Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1. Fernandez-Gonzalez A, La Spada AR, Treadaway J, Higdon JC, Harris BS, Sidman RL, Morgan JI, Zuo J. | 01/21/2010 |