Grant Detail
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Genomic Analysis of Alzheimer's Disease Genes
1 June 1994 - 31 March 2014
NATIONAL INSTITUTE ON AGING
Total Funding: $ 2,446,596
Abnormally aggregates of the protein tau in the form of neurofibrillary tangles (NFT's) and glial tangles are found in a number of neurodegenerative diseases including Alzheimer's disease (AD) and frontotemporal dementia with parkinsonism - chromosome 17 type (FTDP-17). For most of these diseases, the role tau plays in disease initiation and progression is not understood. However, for the autosomal-dominant disorder FTDP-17, mutations in MAPT, the gene that encodes tau protein, cause the disease. The clinical and neuropathologic phenotype produced by different MAPT mutations is highly variable. For some mutations, the initial clinical feature is limited to executive function deterioration. For others, severe behavioral disturbances such as disinhibition or psychosis are the initial symptoms. Different neuropathologic phenotypes are observed. For some mutations, aggregated tau is only seen in neurons as NFT's. For others, both neuronal and glial aggregates are found (glial fibdllary tangles, GFT's). The regional distribution of pathology is also varied. In some cases, pathology is seen broadly distributed in the frontotemporal lobes while in other cases, only brain stem regions are involved as seen in progressive supra nuclear palsy (PSP). Two types of FTDP-17 mutations are known. One type is missense mutations that act at the protein level. The second type of mutations, which is the focus of this application, alter the regulation of the alternative splicing of tau exon 10 (El0). Also, susceptibility to PSP is caused by an unidentified allele(s) at a MAPT polymorphic site(s) that influences MAPTsplicing. For FTDP-17 mutations, phenotypic variability is potentially dependent on differential expression patterns of trans-acting splicing regulatory factors in different subpopulations of neurons and glial cells. Differential phosphorylation of trans-acting factors in different cell types may also be involved in phenotypic vadabUity. In this proposal, we will identify the cis-acting sequence elements in tau that affect the regulation of alternative splicing. We will also identify the trans-factors that interact with the cis-acting elements. Identification of c/s-elements may lead to therapeutic approaches based on evolving strategies for manipulating RNA in vivo. Likewise, identification of trans-factors may lead to protein targets such as kinases that are also therapeutic targets.
26 Resulting Publications
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1.
2012Giovanni Coppola; Subashchandrabose Chinnathambi; Jason JiYong Lee; Beth A Dombroski; Matt C Baker; Alexandra I Soto-Ortolaza; Suzee E Lee; Eric Klein; Alden Y Huang; Renee Sears; et al.
Human molecular genetics 2012;21(15):3500-12. -
2.
2011Günter U Höglinger; Nadine M Melhem; Dennis W Dickson; Patrick M A Sleiman; Li-San Wang; Lambertus Klei; Rosa Rademakers; Rohan de Silva; Irene Litvan; David E Riley; et al.
Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy.
Nature genetics 2011;43(7):699-705. -
3.
2010H L Melrose; J C Dächsel; B Behrouz; S J Lincoln; M Yue; K M Hinkle; C B Kent; E Korvatska; J P Taylor; L Witten; et al.
Neurobiology of disease 2010;40(3):503-17. -
4.
2010D M Kay; C F Stevens; T H Hamza; J S Montimurro; C P Zabetian; S A Factor; A Samii; A Griffith; J W Roberts; E S Molho; et al.
A comprehensive analysis of deletions, multiplications, and copy number variations in PARK2.
Neurology 2010;75(13):1189-94. -
5.
2009Weiva Sieh; Yoonha Choi; Nicola H Chapman; Ulla-Katrina Craig; Ellen J Steinbart; Joseph H Rothstein; Kiyomitsu Oyanagi; Ralph M Garruto; Thomas D Bird; Douglas R Galasko; et al.
Human molecular genetics 2009;18(19):3725-38. -
6.
2008Pamela McMillan; Elena Korvatska; Parvoneh Poorkaj; Zana Evstafjeva; Linda Robinson; Lynne Greenup; James Leverenz; Gerard D Schellenberg; Ian D'Souza
Tau isoform regulation is region- and cell-specific in mouse brain.
The Journal of comparative neurology 2008;511(6):788-803. -
7.
2007Weiva Sieh; Chang-En Yu; Thomas D Bird; Gerard D Schellenberg; Ellen M Wijsman
Human heredity 2007;63(1):26-34. -
8.
2006Ian D'Souza; Gerard D Schellenberg
The Journal of biological chemistry 2006;281(5):2460-9. -
9.
2005Ellen M Wijsman; E Warwick Daw; Xuesong Yu; Ellen J Steinbart; David Nochlin; Thomas D Bird; Gerard D Schellenberg
APOE and other loci affect age-at-onset in Alzheimer's disease families with PS2 mutation.
American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics 2005;132B(1):14-20. -
10.
2004Ellen M Wijsman; E Warwick Daw; Change-En Yu; Haydeh Payami; Ellen J Steinbart; David Nochlin; Erin M Conlon; Thomas D Bird; Gerard D Schellenberg
Evidence for a novel late-onset Alzheimer disease locus on chromosome 19p13.2.
American journal of human genetics 2004;75(3):398-409. -
11.
2003Jason T Huse; Damani Byant; Yaxiong Yang; Donald S Pijak; Ian D'Souza; James J Lah; Virginia M-Y Lee; Robert W Doms; David G Cook
The Journal of biological chemistry 2003;278(19):17141-9. -
12.
2002Parvoneh Poorkaj; Nancy A Muma; Victoria Zhukareva; Elizabeth J Cochran; Kathleen M Shannon; Howard Hurtig; William C Koller; Thomas D Bird; John Q Trojanowski; Virginia M-Y Lee; et al.
An R5L tau mutation in a subject with a progressive supranuclear palsy phenotype.
Annals of neurology 2002;52(4):511-6. -
13.
2002Ian D'Souza; Gerard D Schellenberg
The Journal of biological chemistry 2002;277(29):26587-99. -
14.
2001P Poorkaj; A Kas; I D'Souza; Y Zhou; Q Pham; M Stone; M V Olson; G D Schellenberg
A genomic sequence analysis of the mouse and human microtubule-associated protein tau.
Mammalian genome : official journal of the International Mammalian Genome Society 2001;12(9):700-12. -
15.
2000P Poorkaj; K R Peterson; G D Schellenberg
Single-step conversion of P1 and P1 artificial chromosome clones into yeast artificial chromosomes.
Genomics 2000;68(1):106-10. -
16.
2000I D'Souza; G D Schellenberg
The Journal of biological chemistry 2000;275(23):17700-9. -
17.
2000M Yasuda; J Takamatsu; I D'Souza; R A Crowther; T Kawamata; M Hasegawa; H Hasegawa; M G Spillantini; S Tanimukai; P Poorkaj; et al.
Annals of neurology 2000;47(4):422-9. -
18.
2000G D Schellenberg; I D'Souza; P Poorkaj
The genetics of Alzheimer's disease.
Current psychiatry reports 2000;2(2):158-64. -
19.
1999I D'Souza; P Poorkaj; M Hong; D Nochlin; V M Lee; T D Bird; G D Schellenberg
Proceedings of the National Academy of Sciences of the United States of America 1999;96(10):5598-603. -
20.
1998V Sharma; P Poorkaj; F Hisama; L Bonnycastle; C E Yu; H Massa; B Trask; K P Clancy; D Patterson; S M Weissman; et al.
An expression map from human chromosome 14q24.3.
Genomics 1998;47(2):314-8. -
21.
1998E Levy-Lahad; D Tsuang; T D Bird
Recent advances in the genetics of Alzheimer's disease.
Journal of geriatric psychiatry and neurology 1998;11(2):42-54. -
22.
1996E Levy-Lahad; T D Bird
Genetic factors in Alzheimer's disease: a review of recent advances.
Annals of neurology 1996;40(6):829-40. -
23.
1996M Ikeda; V Sharma; S M Sumi; E A Rogaeva; P Poorkaj; R Sherrington; L Nee; T Tsuda; N Oda; M Watanabe; et al.
The clinical phenotype of two missense mutations in the presenilin I gene in Japanese patients.
Annals of neurology 1996;40(6):912-7. -
24.
1996T D Bird; E Levy-Lahad; P Poorkaj; V Sharma; E Nemens; A Lahad; T H Lampe; G D Schellenberg
Wide range in age of onset for chromosome 1--related familial Alzheimer's disease.
Annals of neurology 1996;40(6):932-6. -
25.
1995G D Schellenberg
Genetic dissection of Alzheimer disease, a heterogeneous disorder.
Proceedings of the National Academy of Sciences of the United States of America 1995;92(19):8552-9. -
26.
1994G D Schellenberg
Alzheimer's disease: implications of genetic studies.
Neurobiology of aging 1994;15 Suppl 2():S141-4.
Scientific Context
This section shows information that has been computed by using the fingerprint of the grant, including related publications, related experts and related grants - all with fingerprints representing significant amounts of overlap between their fingerprint and this grant. The red dots indicate whether those experts or terms actually appear within this grant, showing potential and actual connections.
Related Grants
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1.
IQBAL, KHALID
Subgroups of Alzheimer Disease
15 May 2007 - 30 April 2012
NATIONAL INSTITUTE ON AGING
Total Funding: $ 1,630,946
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2.
LEE, VIRGINIA M
Frontotemporal Dementias: Genotypes and Phenotypes
15 March 2000 - 29 February 2016
NATIONAL INSTITUTE ON AGING
Total Funding: $ 21,445,858
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3.
MILLER, BRUCE L
Frontotemporal Dementia: Genes, Images, and Emotions
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NATIONAL INSTITUTE ON AGING
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Related Publications
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1.
2002Luc Buée; Malika Hamdane; Patrice Delobel; Anne-Véronique Sambo; Séverine Bégard; Antoine Ghestem; Nicolas Sergeant; André Delacourte
[Tau story: from frontotemporal dementia to other tauopathies].
Journal de la Société de biologie 2002;196(1):103-8. -
2.
2000Z Jiang; J Cote; J M Kwon; A M Goate; J Y Wu
Molecular and cellular biology 2000;20(11):4036-48. -
3.
1998M G Spillantini; M Goedert
Tau protein pathology in neurodegenerative diseases.
Trends in neurosciences 1998;21(10):428-33.
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