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Items: 38


The role of neurofilament aggregation in neurodegeneration: lessons from rare inherited neurological disorders.

Didonna A, Opal P.

Mol Neurodegener. 2019 May 16;14(1):19. doi: 10.1186/s13024-019-0318-4. Review.


Self-assembling vascular endothelial growth factor nanoparticles improve function in spinocerebellar ataxia type 1.

Hu YS, Do J, Edamakanti CR, Kini AR, Martina M, Stupp SI, Opal P.

Brain. 2019 Feb 1;142(2):312-321. doi: 10.1093/brain/awy328.


Loss of the dystonia gene Thap1 leads to transcriptional deficits that converge on common pathogenic pathways in dystonic syndromes.

Frederick NM, Shah PV, Didonna A, Langley MR, Kanthasamy AG, Opal P.

Hum Mol Genet. 2019 Apr 15;28(8):1343-1356. doi: 10.1093/hmg/ddy433.


Trinucleotide Repeat Expansion Diseases, RNAi, and Cancer.

Murmann AE, Yu J, Opal P, Peter ME.

Trends Cancer. 2018 Oct;4(10):684-700. doi: 10.1016/j.trecan.2018.08.004. Epub 2018 Sep 26. Review.


Mutant ataxin1 disrupts cerebellar development in spinocerebellar ataxia type 1.

Edamakanti CR, Do J, Didonna A, Martina M, Opal P.

J Clin Invest. 2018 Jun 1;128(6):2252-2265. doi: 10.1172/JCI96765. Epub 2018 Apr 23.


ANP32A regulates histone H3 acetylation and promotes leukemogenesis.

Yang X, Lu B, Sun X, Han C, Fu C, Xu K, Wang M, Li D, Chen Z, Opal P, Wen Q, Crispino JD, Wang QF, Huang Z.

Leukemia. 2018 Jul;32(7):1587-1597. doi: 10.1038/s41375-018-0010-7. Epub 2018 Feb 2.


ANP32A dysregulation contributes to abnormal megakaryopoiesis in acute megakaryoblastic leukemia.

Sun X, Lu B, Han C, Qiu W, Jin Q, Li D, Li Q, Yang Q, Wen Q, Opal P, Kini AR, Crispino JD, Huang Z.

Blood Cancer J. 2017 Dec 22;7(12):661. doi: 10.1038/s41408-017-0031-x. No abstract available.


Spinocerebellar Ataxia Type 1.

Opal P, Ashizawa T.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.
1998 Oct 1 [updated 2017 Jun 22].


The role of gigaxonin in the degradation of the glial-specific intermediate filament protein GFAP.

Lin NH, Huang YS, Opal P, Goldman RD, Messing A, Perng MD.

Mol Biol Cell. 2016 Dec 15;27(25):3980-3990. Epub 2016 Oct 26.


Intermediate filament aggregates cause mitochondrial dysmotility and increase energy demands in giant axonal neuropathy.

Israeli E, Dryanovski DI, Schumacker PT, Chandel NS, Singer JD, Julien JP, Goldman RD, Opal P.

Hum Mol Genet. 2016 Jun 1;25(11):2143-2157. Epub 2016 Mar 21.


Advances in Sequencing Technologies for Understanding Hereditary Ataxias: A Review.

Didonna A, Opal P.

JAMA Neurol. 2016 Dec 1;73(12):1485-1490. doi: 10.1001/jamaneurol.2016.3097. Review.


Paraneoplastic cerebellar degeneration with anti-Yo antibodies - a review.

Venkatraman A, Opal P.

Ann Clin Transl Neurol. 2016 Jun 30;3(8):655-63. doi: 10.1002/acn3.328. eCollection 2016 Aug. Review.


Mutant Ataxin-1 Inhibits Neural Progenitor Cell Proliferation in SCA1.

Cvetanovic M, Hu YS, Opal P.

Cerebellum. 2017 Apr;16(2):340-347. doi: 10.1007/s12311-016-0794-9.


Abnormal intermediate filament organization alters mitochondrial motility in giant axonal neuropathy fibroblasts.

Lowery J, Jain N, Kuczmarski ER, Mahammad S, Goldman A, Gelfand VI, Opal P, Goldman RD.

Mol Biol Cell. 2016 Feb 15;27(4):608-16. doi: 10.1091/mbc.E15-09-0627. Epub 2015 Dec 23.


Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability.

Dell'Orco JM, Wasserman AH, Chopra R, Ingram MA, Hu YS, Singh V, Wulff H, Opal P, Orr HT, Shakkottai VG.

J Neurosci. 2015 Aug 12;35(32):11292-307. doi: 10.1523/JNEUROSCI.1357-15.2015.


The promise and perils of HDAC inhibitors in neurodegeneration.

Didonna A, Opal P.

Ann Clin Transl Neurol. 2015 Jan;2(1):79-101. doi: 10.1002/acn3.147. Epub 2014 Dec 3. Review.


Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.

Cvetanovic M, Ingram M, Orr H, Opal P.

Neuroscience. 2015 Mar 19;289:289-99. doi: 10.1016/j.neuroscience.2015.01.003. Epub 2015 Jan 14.


An investigation of diffusion imaging techniques in the evaluation of spinocerebellar ataxia and multisystem atrophy.

Rozenfeld MN, Nemeth AJ, Walker MT, Mohan P, Wang X, Parrish TB, Opal P.

J Clin Neurosci. 2015 Jan;22(1):166-72. doi: 10.1016/j.jocn.2014.08.006. Epub 2014 Nov 26.


The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1.

Venkatraman A, Hu YS, Didonna A, Cvetanovic M, Krbanjevic A, Bilesimo P, Opal P.

Hum Mol Genet. 2014 Jul 15;23(14):3733-45. doi: 10.1093/hmg/ddu081. Epub 2014 Mar 4.


Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation.

Mahammad S, Murthy SN, Didonna A, Grin B, Israeli E, Perrot R, Bomont P, Julien JP, Kuczmarski E, Opal P, Goldman RD.

J Clin Invest. 2013 May;123(5):1964-75. doi: 10.1172/JCI66387. Epub 2013 Apr 15.


Explaining intermediate filament accumulation in giant axonal neuropathy.

Opal P, Goldman RD.

Rare Dis. 2013 Jun 17;1:e25378. doi: 10.4161/rdis.25378. eCollection 2013.


LANP mediates neuritic pathology in Spinocerebellar ataxia type 1.

Cvetanovic M, Kular RK, Opal P.

Neurobiol Dis. 2012 Dec;48(3):526-32. doi: 10.1016/j.nbd.2012.07.024. Epub 2012 Aug 4.


Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1.

Cvetanovic M, Patel JM, Marti HH, Kini AR, Opal P.

Nat Med. 2011 Oct 16;17(11):1445-7. doi: 10.1038/nm.2494.


Cpd-1 null mice display a subtle neurological phenotype.

Kular RK, Gogliotti RG, Opal P.

PLoS One. 2010 Sep 9;5(9). pii: e12649. doi: 10.1371/journal.pone.0012649.


Neuronal differentiation is regulated by leucine-rich acidic nuclear protein (LANP), a member of the inhibitor of histone acetyltransferase complex.

Kular RK, Cvetanovic M, Siferd S, Kini AR, Opal P.

J Biol Chem. 2009 Mar 20;284(12):7783-92. doi: 10.1074/jbc.M806150200. Epub 2009 Jan 9.


Association of spinocerebellar ataxia type 3 and spinocerebellar ataxia type 8 microsatellite expansions: genetic counseling implications.

Paganoni S, Seelaus CA, Ormond KE, Opal P.

Mov Disord. 2008 Jan;23(1):154-5. No abstract available.


The role of LANP and ataxin 1 in E4F-mediated transcriptional repression.

Cvetanovic M, Rooney RJ, Garcia JJ, Toporovskaya N, Zoghbi HY, Opal P.

EMBO Rep. 2007 Jul;8(7):671-7. Epub 2007 Jun 8.


Huntington's disease presenting as postsurgical psychosis.

Paganoni S, Naidech AM, Opal P.

Mov Disord. 2007 Jun 15;22(8):1209-10. No abstract available.


In cis autosomal dominant mutation of Senataxin associated with tremor/ataxia syndrome.

Bassuk AG, Chen YZ, Batish SD, Nagan N, Opal P, Chance PF, Bennett CL.

Neurogenetics. 2007 Jan;8(1):45-9. Epub 2006 Nov 10.


Generation and characterization of LANP/pp32 null mice.

Opal P, Garcia JJ, McCall AE, Xu B, Weeber EJ, Sweatt JD, Orr HT, Zoghbi HY.

Mol Cell Biol. 2004 Apr;24(8):3140-9.


Mapmodulin/leucine-rich acidic nuclear protein binds the light chain of microtubule-associated protein 1B and modulates neuritogenesis.

Opal P, Garcia JJ, Propst F, Matilla A, Orr HT, Zoghbi HY.

J Biol Chem. 2003 Sep 5;278(36):34691-9. Epub 2003 Jun 14.


The role of chaperones in polyglutamine disease.

Opal P, Zoghbi HY.

Trends Mol Med. 2002 May;8(5):232-6. Review.


Intrafamilial phenotypic variability of the DYT1 dystonia: from asymptomatic TOR1A gene carrier status to dystonic storm.

Opal P, Tintner R, Jankovic J, Leung J, Breakefield XO, Friedman J, Ozelius L.

Mov Disord. 2002 Mar;17(2):339-45.


Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice.

Cummings CJ, Sun Y, Opal P, Antalffy B, Mestril R, Orr HT, Dillmann WH, Zoghbi HY.

Hum Mol Genet. 2001 Jul 1;10(14):1511-8.


The function of intermediate filaments in cell shape and cytoskeletal integrity.

Goldman RD, Khuon S, Chou YH, Opal P, Steinert PM.

J Cell Biol. 1996 Aug;134(4):971-83.


Intermediate filament dynamics.

Eriksson JE, Opal P, Goldman RD.

Curr Opin Cell Biol. 1992 Feb;4(1):99-104. Review.


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