| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Janghoo Lim, Tong Hao, Chad Shaw, Akash J Patel, Gábor Szabó, Jean-François Rual, C Joseph Fisk, Ning Li, Alex Smolyar, David E Hill, Albert-László Barabási, Marc Vidal, Huda Y Zoghb. A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. Cell. vol 125. issue 4. 2006-07-12. PMID:16713569. |
a protein-protein interaction network for human inherited ataxias and disorders of purkinje cell degeneration. |
2006-07-12 |
2023-08-12 |
human |
| Joy T Walter, Karina Alviña, Mary D Womack, Carolyn Chevez, Kamran Khodakha. Decreases in the precision of Purkinje cell pacemaking cause cerebellar dysfunction and ataxia. Nature neuroscience. vol 9. issue 3. 2006-04-14. PMID:16474392. |
decreases in the precision of purkinje cell pacemaking cause cerebellar dysfunction and ataxia. |
2006-04-14 |
2023-08-12 |
mouse |
| Lihong Zhao, Chantal Longo-Guess, Belinda S Harris, Jeong-Woong Lee, Susan L Ackerma. Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP. Nature genetics. vol 37. issue 9. 2005-09-29. PMID:16116427. |
mice homozygous with respect to the woozy (wz) mutation develop adult-onset ataxia with cerebellar purkinje cell loss. |
2005-09-29 |
2023-08-12 |
mouse |
| Peter L Oliver, Kay E Davie. Analysis of human neurological disorders using mutagenesis in the mouse. Clinical science (London, England : 1979). vol 108. issue 5. 2005-06-14. PMID:15831088. |
four lines are discussed, one displaying late-onset ataxia caused by purkinje cell loss and an allelic series of three tremor mutants suffering from hypomyelination of the peripheral nerve. |
2005-06-14 |
2023-08-12 |
mouse |
| Peter L Oliver, Emmanuelle Bitoun, Joanne Clark, Emma L Jones, Kay E Davie. Mediation of Af4 protein function in the cerebellum by Siah proteins. Proceedings of the National Academy of Sciences of the United States of America. vol 101. issue 41. 2004-12-02. PMID:15459319. |
af4 is mutated in the robotic mouse that is characterized by ataxia and purkinje cell loss. |
2004-12-02 |
2023-08-12 |
mouse |
| Naohiro Yamaguchi, Suehiro Sakaguchi, Kazuto Shigematsu, Nobuhiko Okimura, Shigeru Katamin. Doppel-induced Purkinje cell death is stoichiometrically abrogated by prion protein. Biochemical and biophysical research communications. vol 319. issue 4. 2004-08-02. PMID:15194501. |
mice devoid of prion protein (prp) exhibiting ataxia and purkinje cell degeneration, such as ngsk prnp(-/-) mice, ectopically express prp-like protein, dpl, in neurons including purkinje cells. |
2004-08-02 |
2023-08-12 |
mouse |
| Yosuke Ando, Nobutsune Ichihara, Shigehito Takeshita, Yoshiaki Saito, Takeki Kikuchi, Noboru Wakasug. Histological and ultrastructural features in the early stage of Purkinje cell degeneration in the cerebellar calcification (CC) rat. Experimental animals. vol 53. issue 2. 2004-07-20. PMID:15153669. |
the cerebellar calcification (cc) rat is a new neurodegenerative mutant with severe purkinje cell loss and symmetrical calcifications in the cerebellar cortex manifesting ataxia: lack of coordination in body movements. |
2004-07-20 |
2023-08-12 |
rat |
| F Delis, A Mitsacos, P Giompre. Dopamine receptor and transporter levels are altered in the brain of Purkinje Cell Degeneration mutant mice. Neuroscience. vol 125. issue 1. 2004-06-03. PMID:15051164. |
the purkinje cell degeneration (nna1pcd, pcd) mutant mouse is mainly characterized by the complete, primary loss of the purkinje cells and the secondary, partial, retrograde loss of the granule and inferior olive neurons and is considered a model of human degenerative ataxia. |
2004-06-03 |
2023-08-12 |
mouse |
| Lucy Anderson, Daniela Rossi, Jackie Linehan, Sebastian Brandner, Charles Weissman. Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment. Proceedings of the National Academy of Sciences of the United States of America. vol 101. issue 10. 2004-05-05. PMID:15007176. |
however, ubiquitous dpl overexpression in the brain of prp knockout mice correlated with ataxia and purkinje cell degeneration in the cerebellum. |
2004-05-05 |
2023-08-12 |
mouse |
| Lucy Anderson, Daniela Rossi, Jackie Linehan, Sebastian Brandner, Charles Weissman. Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment. Proceedings of the National Academy of Sciences of the United States of America. vol 101. issue 10. 2004-05-05. PMID:15007176. |
such mice showed ataxia and purkinje cell loss that depended on the level of dpl expression. |
2004-05-05 |
2023-08-12 |
mouse |
| Takayuki Harada, Liliam L Pineda, Akinobu Nakano, Koji Omura, Li Zhou, Masaaki Iijima, Yoji Yamasaki, Minesuke Yokoyam. Ataxia and male sterility (AMS) mouse. A new genetic variant exhibiting degeneration and loss of cerebellar Purkinje cells and spermatic cells. Pathology international. vol 53. issue 6. 2004-03-16. PMID:12787313. |
the cell loss in the purkinje cell layer began before onset of ataxia and rapidly progressed towards near-complete loss by 6 weeks of age. |
2004-03-16 |
2023-08-12 |
mouse |
| Ryuichiro Atarashi, Noriyuki Nishida, Kazuto Shigematsu, Shinji Goto, Takahito Kondo, Suehiro Sakaguchi, Shigeru Katamin. Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration. The Journal of biological chemistry. vol 278. issue 31. 2003-09-10. PMID:12759361. |
a line of prp-deficient mice, ngsk prnp0/0, ectopically expressing prplp/dpl in neurons, exhibits late-onset ataxia because of purkinje cell death that is prevented by a transgene encoding wild-type mouse prp. |
2003-09-10 |
2023-08-12 |
mouse |
| Eckhard Flechsig, Ivan Hegyi, Rainer Leimeroth, Armando Zuniga, Daniela Rossi, Antonio Cozzio, Petra Schwarz, Thomas Rülicke, Jürgen Götz, Adriano Aguzzi, Charles Weissman. Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia. The EMBO journal. vol 22. issue 12. 2003-08-19. PMID:12805223. |
expression of truncated prp targeted to purkinje cells of prp knockout mice causes purkinje cell death and ataxia. |
2003-08-19 |
2023-08-12 |
mouse |
| A M Ghoumari, I Dusart, M El-Etr, F Tronche, C Sotelo, M Schumacher, E-E Baulie. Mifepristone (RU486) protects Purkinje cells from cell death in organotypic slice cultures of postnatal rat and mouse cerebellum. Proceedings of the National Academy of Sciences of the United States of America. vol 100. issue 13. 2003-07-31. PMID:12810951. |
indeed, this steroid is able to prevent purkinje cells from death in organotypic cultures of cerebellar slices from purkinje cell degeneration (pcd) mutant mice, a murine model of hereditary neurodegenerative ataxia. |
2003-07-31 |
2023-08-12 |
mouse |
| Thasia Leimig, Linda Mann, Maria del Pilar Martin, Erik Bonten, Derek Persons, James Knowles, James A Allay, John Cunningham, Arthur W Nienhuis, Richard Smeyne, Alessandra d'Azz. Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells. Blood. vol 99. issue 9. 2002-06-19. PMID:11964280. |
however, ppca expression in the central nervous system was apparently sufficient to delay the onset of purkinje cell degeneration and to correct the ataxia. |
2002-06-19 |
2023-08-12 |
mouse |
| Pamela J Skinner, Cynthia A Vierra-Green, Effat Emamian, Huda Y Zoghbi, Harry T Or. Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice. Neuromolecular medicine. vol 1. issue 1. 2002-06-14. PMID:12025814. |
transgenic mice that overexpress a mutant allele of the sca1 gene develop a progressive ataxia and purkinje cell pathology. |
2002-06-14 |
2023-08-12 |
mouse |
| R C Moore, P Mastrangelo, E Bouzamondo, C Heinrich, G Legname, S B Prusiner, L Hood, D Westaway, S J DeArmond, P Trembla. Doppel-induced cerebellar degeneration in transgenic mice. Proceedings of the National Academy of Sciences of the United States of America. vol 98. issue 26. 2002-01-22. PMID:11734625. |
in contrast, tg(dpl)zrchi mice showed cerebellar granule and purkinje cell loss; the age of onset of ataxia was inversely proportional to the levels of dpl protein. |
2002-01-22 |
2023-08-12 |
mouse |
| B Schröder, B Franz, P Hempfling, M Selbert, T Jürgens, H A Kretzschmar, M Bodemer, S Poser, I Zer. Polymorphisms within the prion-like protein gene (Prnd) and their implications in human prion diseases, Alzheimer's disease and other neurological disorders. Human genetics. vol 109. issue 3. 2001-12-07. PMID:11702213. |
recently, the murine prp-like protein doppel (dpl) was described and was shown to be overexpressed in certain strains of prp knockout mice and to cause neurological diseases such as ataxia and purkinje cell loss. |
2001-12-07 |
2023-08-12 |
mouse |
| P J Skinner, C A Vierra-Green, H B Clark, H Y Zoghbi, H T Or. Altered trafficking of membrane proteins in purkinje cells of SCA1 transgenic mice. The American journal of pathology. vol 159. issue 3. 2001-10-11. PMID:11549583. |
overexpression of mutant ataxin-1 in purkinje cells of transgenic mice results in a progressive ataxia and purkinje cell pathology that are very similar to those seen in sca1 patients. |
2001-10-11 |
2023-08-12 |
mouse |
| W F Kaemmerer, C M Rodrigues, C J Steer, W C Lo. Creatine-supplemented diet extends Purkinje cell survival in spinocerebellar ataxia type 1 transgenic mice but does not prevent the ataxic phenotype. Neuroscience. vol 103. issue 3. 2001-06-21. PMID:11274790. |
we tested the effects of a 2% creatine-supplemented diet and treatment with taurine-conjugated ursodeoxycholic acid, a bile constituent that can inhibit the mitochondrial permeability transition, on ataxia and purkinje cell survival in a transgenic model of spinocerebellar ataxia type 1. |
2001-06-21 |
2023-08-12 |
mouse |