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Wyświetlanie 1-6 z 6
Tytuł :
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice.
Autorzy :
Cummings CJ; Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA.
Sun Y
Opal P
Antalffy B
Mestril R
Orr HT
Dillmann WH
Zoghbi HY
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Źródło :
Human molecular genetics [Hum Mol Genet] 2001 Jul 01; Vol. 10 (14), pp. 1511-8.
Typ publikacji :
Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Motor Activity*
HSP70 Heat-Shock Proteins/*genetics
Nerve Tissue Proteins/*genetics
Neurodegenerative Diseases/*prevention & control
Nuclear Proteins/*genetics
Protein Structure, Tertiary/*genetics
Trinucleotide Repeats/*genetics
Animals ; Ataxin-1 ; Ataxins ; Brain Stem/pathology ; Cerebellum/pathology ; Gene Expression ; In Vitro Techniques ; Inclusion Bodies/genetics ; Inclusion Bodies/pathology ; Mice ; Mice, Transgenic ; Neurodegenerative Diseases/pathology ; Neurons/pathology ; Protein Conformation ; Purkinje Cells/pathology
Czasopismo naukowe
Tytuł :
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.
Autorzy :
Cummings CJ; Program in Cell and Molecular Biology, Baylor College of Medicine, Houston, Texas 77030, USA.
Reinstein E
Sun Y
Antalffy B
Jiang Y
Ciechanover A
Orr HT
Beaudet AL
Zoghbi HY
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Źródło :
Neuron [Neuron] 1999 Dec; Vol. 24 (4), pp. 879-92.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Cell Nucleus/*genetics
Inclusion Bodies/*genetics
Ligases/*genetics
Nerve Tissue Proteins/*genetics
Nuclear Proteins/*genetics
Peptides/*toxicity
Spinocerebellar Degenerations/*genetics
Animals ; Ataxin-1 ; Ataxins ; Cell Nucleus/pathology ; Cells, Cultured ; Cysteine Endopeptidases/metabolism ; Fluorescent Antibody Technique ; HeLa Cells ; Humans ; Immunoblotting ; Immunohistochemistry ; Inclusion Bodies/pathology ; Ligases/deficiency ; Mice ; Mice, Knockout ; Microscopy, Confocal ; Multienzyme Complexes/metabolism ; Mutation/physiology ; Nerve Tissue Proteins/biosynthesis ; Nuclear Proteins/biosynthesis ; Phenotype ; Plasmids/genetics ; Proteasome Endopeptidase Complex ; Purkinje Cells/metabolism ; Purkinje Cells/pathology ; Purkinje Cells/ultrastructure ; Spinocerebellar Degenerations/pathology ; Ubiquitin-Protein Ligases ; Ubiquitins/genetics ; Ubiquitins/metabolism
Czasopismo naukowe
Tytuł :
Progress in pathogenesis studies of spinocerebellar ataxia type 1.
Autorzy :
Cummings CJ; Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA.
Orr HT
Zoghbi HY
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Źródło :
Philosophical transactions of the Royal Society of London. Series B, Biological sciences [Philos Trans R Soc Lond B Biol Sci] 1999 Jun 29; Vol. 354 (1386), pp. 1079-81.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
MeSH Terms :
Nerve Tissue Proteins/*genetics
Nuclear Proteins/*genetics
Spinocerebellar Degenerations/*genetics
Spinocerebellar Degenerations/*pathology
Animals ; Ataxin-1 ; Ataxins ; Brain Stem/pathology ; Cerebellum/pathology ; HeLa Cells ; Humans ; Inclusion Bodies/genetics ; Inclusion Bodies/pathology ; Mice ; Mice, Transgenic ; Peptides/genetics ; Purkinje Cells/pathology ; Spinocerebellar Degenerations/physiopathology ; Transfection ; Trinucleotide Repeats
Czasopismo naukowe
Tytuł :
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.
Autorzy :
Cummings CJ; Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
Mancini MA
Antalffy B
DeFranco DB
Orr HT
Zoghbi HY
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Źródło :
Nature genetics [Nat Genet] 1998 Jun; Vol. 19 (2), pp. 148-54.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Protein Folding*
Cysteine Endopeptidases/*metabolism
Molecular Chaperones/*physiology
Multienzyme Complexes/*metabolism
Nerve Tissue Proteins/*metabolism
Nuclear Proteins/*metabolism
Spinocerebellar Degenerations/*pathology
Animals ; Ataxin-1 ; Ataxins ; Carrier Proteins/metabolism ; Cells, Cultured ; HSC70 Heat-Shock Proteins ; HSP40 Heat-Shock Proteins ; HSP70 Heat-Shock Proteins/metabolism ; HeLa Cells ; Heat-Shock Proteins/metabolism ; Humans ; Mice ; Mice, Transgenic ; Nerve Tissue Proteins/genetics ; Nuclear Proteins/genetics ; Proteasome Endopeptidase Complex ; Protein Conformation ; Purkinje Cells/metabolism ; Purkinje Cells/pathology ; Spinocerebellar Degenerations/genetics ; Transfection
Czasopismo naukowe
Tytuł :
The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1.
Autorzy :
Matilla A; Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA.
Koshy BT
Cummings CJ
Isobe T
Orr HT
Zoghbi HY
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Źródło :
Nature [Nature] 1997 Oct 30; Vol. 389 (6654), pp. 974-8.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Cerebellum/*metabolism
Nerve Tissue Proteins/*metabolism
Neuropeptides/*metabolism
Nuclear Proteins/*metabolism
Animals ; Ataxin-1 ; Ataxins ; Binding Sites ; COS Cells ; Cell Nucleus/metabolism ; Cerebellum/cytology ; Cloning, Molecular ; Glutamine/metabolism ; Humans ; Immunohistochemistry ; Mice ; Microscopy, Confocal ; Mutation ; Nerve Tissue Proteins/genetics ; Nuclear Matrix/metabolism ; Nuclear Proteins/genetics ; Purkinje Cells/metabolism ; Rats ; Saccharomyces cerevisiae ; Spinocerebellar Degenerations/metabolism
Czasopismo naukowe
Tytuł :
Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures.
Autorzy :
Skinner PJ; Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis 55455, USA.
Koshy BT
Cummings CJ
Klement IA
Helin K
Servadio A
Zoghbi HY
Orr HT
Pokaż więcej
Źródło :
Nature [Nature] 1997 Oct 30; Vol. 389 (6654), pp. 971-4.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Glutamine/*physiology
Nerve Tissue Proteins/*physiology
Nuclear Matrix/*pathology
Nuclear Proteins/*physiology
Spinocerebellar Degenerations/*pathology
Animals ; Ataxin-1 ; Ataxins ; Brain/metabolism ; Brain/pathology ; COS Cells ; Glutamine/chemistry ; Humans ; Immunohistochemistry ; Mice ; Mice, Transgenic ; Mutation ; Nerve Tissue Proteins/chemistry ; Nerve Tissue Proteins/genetics ; Nuclear Proteins/chemistry ; Nuclear Proteins/genetics ; Purkinje Cells/metabolism ; Purkinje Cells/pathology ; Spinocerebellar Degenerations/etiology ; Spinocerebellar Degenerations/genetics
Czasopismo naukowe
    Wyświetlanie 1-6 z 6

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