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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 19 Dec 2016.

ATP/GTP binding protein 1

Purkinje cell degeneration
Top mentioned proteins: AGE, CAN, GIRK2, ACID, HAD
Papers on Purkinje cell degeneration
Linking Essential Tremor to the Cerebellum-Animal Model Evidence.
Handforth, Los Angeles, United States. In Cerebellum, Jan 2016
We describe models in which chronic partial PC loss is associated with tremor, such as the Weaver mouse, and others with PC loss that do not show tremor, such as the Purkinje cell degeneration mouse.
Cerebellar Atrophy in Cortical Myoclonic Tremor and Not in Hereditary Essential Tremor-a Voxel-Based Morphometry Study.
van Rootselaar et al., Amsterdam, Netherlands. In Cerebellum, Nov 2015
Familial cortical myoclonic tremor with epilepsy (FCMTE), with evident Purkinje cell degeneration, can be an ET mimic.
Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain.
Mao et al., Stony Brook, United States. In Plos Genet, Oct 2015
Acer3 knockout causes an age-dependent accumulation of various ceramides and C18:1-monohexosylceramide and abolishes the age-related increase in the levels of sphingosine and S1P in the brain; thereby resulting in Purkinje cell degeneration in the cerebellum and deficits in motor coordination and balance.
Transplantation and Stem Cell Therapy for Cerebellar Degenerations.
Cendelin, Plzeň, Czech Republic. In Cerebellum, Aug 2015
By now, transplantation has been shown to ameliorate cerebellar function, e.g. in Purkinje cell degeneration mice, Lurcher mutant mice and mouse models of spinocerebellar ataxia type 1 and type 2 and Niemann-Pick disease type C. Despite the lack of direct comparative studies, it appears that there might be differences in graft development and functioning between various types of cerebellar degeneration.
The autosomal dominant spinocerebellar ataxias: emerging mechanistic themes suggest pervasive Purkinje cell vulnerability.
Gomez et al., Chicago, United States. In J Neurol Neurosurg Psychiatry, May 2015
The spinocerebellar ataxias are a genetically heterogeneous group of disorders with clinically overlapping phenotypes arising from Purkinje cell degeneration, cerebellar atrophy and varying degrees of degeneration of other grey matter regions.
Lack of Cytosolic Carboxypeptidase 1 Leads to Subfertility due to the Reduced Number of Antral Follicles in pcd3J-/- Females.
Park et al., Seoul, South Korea. In Plos One, 2014
Females homozygous for the Purkinje cell degeneration mutation (pcd) are fertile, although the success rate is much lower than in the wild type.
A quantitative framework for whole-body coordination reveals specific deficits in freely walking ataxic mice.
Carey et al., Lisbon, Portugal. In Elife, 2014
Analysis of visibly ataxic Purkinje cell degeneration (pcd) mice reveals that while differences in the forward motion of individual paws are fully accounted for by changes in walking speed and body size, more complex 3D trajectories and, especially, inter-limb and whole-body coordination are specifically impaired.
High ω3-polyunsaturated fatty acids in fat-1 mice prevent streptozotocin-induced Purkinje cell degeneration through BDNF-mediated autophagy.
Kim et al., Taejŏn, South Korea. In Sci Rep, 2014
We evaluated whether fat-1 transgenic mice, a well-established animal model that endogenously synthesizes ω3 polyunsaturated fatty acids (ω3-PUFA), are protected from Purkinje cell degeneration in streptozotocin (STZ)-treated model with fat-1 mice.
From mice to men: lessons from mutant ataxic mice.
Cendelin, Plzeň, Czech Republic. In Cerebellum Ataxias, 2013
Lurcher, Hot-foot, Purkinje cell degeneration, Nervous, Staggerer, Weaver, Reeler, and Scrambler mouse models and mouse models of SCA1, SCA2, SCA3, SCA6, SCA7, SCA23, DRPLA, Niemann-Pick disease and Friedreich ataxia are reviewed with special regard to cerebellar pathology, pathogenesis, functional changes and possible therapeutic influences, if any.
Cytosolic carboxypeptidase 1 is involved in processing α- and β-tubulin.
Fricker et al., New York City, United States. In J Biol Chem, 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.
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.
Alonso et al., Salamanca, Spain. In Neuroscience, 2012
These data indicated that serotonergic function in the MOB is closely related to olfactory activity and that mitral cell loss induces serotonergic plastic responses.
Spinocerebellar ataxia type 5.
Ranum et al., Minneapolis, United States. In Handb Clin Neurol, 2011
Consistent with Purkinje cell degeneration in SCA5, β-III spectrin is highly expressed in cerebellar Purkinje cells.
Abnormal sperm development in pcd(3J)-/- mice: the importance of Agtpbp1 in spermatogenesis.
Park et al., Seoul, South Korea. In Mol Cells, 2011
Agtpbp1 plays an important role in spermatogenesis and is important for survival of germ cells at spermatocytes stage onward
A family of protein-deglutamylating enzymes associated with neurodegeneration.
Janke et al., Montpellier, France. In Cell, 2010
Study analyzes Purkinje cell degeneration (pcd) mice that lack functional CCP1 and shows that microtubule hyperglutamylation is directly linked to neurodegeneration.
Nna1 mediates Purkinje cell dendritic development via lysyl oxidase propeptide and NF-κB signaling.
Sidman et al., Boston, United States. In Neuron, 2010
This study provided insight into Nna1's role in neuronal development and why its absence renders Purkinje cells more vulnerable.
Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28.
Taroni et al., Milano, Italy. In Nat Genet, 2010
Autosomal dominant spinocerebellar ataxias (SCAs) are genetically heterogeneous neurological disorders characterized by cerebellar dysfunction mostly due to Purkinje cell degeneration.
A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration.
Zoghbi et al., Houston, United States. In Cell, 2006
Many human inherited neurodegenerative disorders are characterized by loss of balance due to cerebellar Purkinje cell (PC) degeneration.
The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins.
Zoghbi et al., Houston, United States. In Cell, 2005
These results indicate that the Atx-1/Gfi-1 interaction contributes to the selective Purkinje cell degeneration in SCA1.
Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1.
Zuo et al., Memphis, United States. In Science, 2002
identifed Nna1 as the gene mutated in the original Purkinje cell degeneration(pcd)and two additional pcd alleles (pcd2J and pcd3J)
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