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Immunofluorescence analysis of Ataxin 7 was performed using 70% confluent log phase U-87 MG cells treated with 100 ng of Nocodazole for 16 hours. The cells were fixed with 4% paraformaldehyde for 10 minutes, permeabilized with 0.1% Triton™ X-100 for 10 minutes, and blocked with 1% BSA for 1 hour at room temperature. The cells were labeled with Ataxin 7 Rabbit Polyclonal Antibody (PA1-749) at 2 µg/ml in 0.1% BSA and incubated for 3 hours at room temperature and then labeled with Goat anti-Rabbit IgG (H+L) Superclonal™ Secondary Antibody, Alexa Fluor® 488 conjugate (A27034) a dilution of 1:2000 for 45 minutes at room temperature (Panel a: green). Nuclei (Panel b: blue) were stained with SlowFade® Gold Antifade Mountant with DAPI (S36938). F-actin (Panel c: red) was stained with Alexa Fluor® 555 Rhodamine Phalloidin (Product # R415, 1:300). Panel d represents the merged image showing cytoplasmic localization. Panel e is untreated cell with nuclear localization. Panel f represents control cells with no primary antibody to assess background. The images were captured at 60X magnification..
|Tested species reactivity||Fruit fly, Human|
|Published species reactivity||Rat, Fruit fly, Human, Mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||Synthetic Peptide: M(1) S E R A A D D V R G E P R R A A(17) C|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS with 1mg/ml BSA|
|Contains||0.05% sodium azide|
|Storage Conditions||-20° C, Avoid Freeze/Thaw Cycles|
|Tested Applications||Dilution *|
|Flow Cytometry (Flow)||3-5 µg/10^6 cells|
|Immunocytochemistry (ICC)||2 µg/ml|
|Immunofluorescence (IF)||2 µg/ml|
|Immunohistochemistry (Frozen) (IHC (F))||Assay dependent|
|Western Blot (WB)||1 ug/ml|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
PA1-749 detects ataxin 7 protein from transfected cell lysate.
PA1-749 has successfully been used in immunohistochemistry and Western blot procedures. By Western blot, this antibody detects an ~96 kDa protein representing human ataxin 7 in transfected COS-1 cells.
The PA1-749 immunizing peptide corresponds to amino acid residues 1-17 from human ataxin 7. This sequence is completely conserved in mouse ataxin 7. This peptide (Cat. # PEP-217) is available for use in neutralization and control experiments.
Spinocerebellar ataxia type 7 (SCA7) belongs to a class of hereditary neurodegenrative diseases characterized by abnormally expanded polyglutamine (CAG) repeats at the N-terminus. This autosomal dominant cerebellar ataxia primarily affects the cerebellum, retina, and brain stem and causes dementia, macular degeneration and other neurodegenerative characteristics.
SCA7 gene encodes the ubiquitous 892 residue protein ataxin-7. This protein is typically located in the cytoplasm and on the nuclear membrane of normal brain neurons. In cells where there is a mutation of the SCA7 gene, ataxin 7 accumulates in intranuclear inclusions and can result in cell death.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.
PA1-749 was used in immunohistochemistry - free floating and western blot to test if reducing ataxin-7 in Purkinje cells is beneficial in an animal model of SCA7
|Ramachandran PS,Boudreau RL,Schaefer KA,La Spada AR,Davidson BL||Molecular therapy : the journal of the American Society of Gene Therapy (22:1635)||2014|
RNA interference-based therapy for spinocerebellar ataxia type 7 retinal degeneration.
PA1-749 was used in western blot to study the beneficial retinal effects of RNAi-mediated knockdown of ataxin-7 in spinocerebellar ataxia type 7
|Ramachandran PS,Bhattarai S,Singh P,Boudreau RL,Thompson S,Laspada AR,Drack AV,Davidson BL||PloS one (9:null)||2014|
Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model.
PA1-749 was used in western blot to study the roles of HDAC3 and ataxin-7 in a murine model of spinocerebellar ataxia type 7
|Duncan CE,An MC,Papanikolaou T,Rugani C,Vitelli C,Ellerby LM||Molecular neurodegeneration (8:null)||2014|
Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation.
PA1-749 was used in western blot to study the relationship between ataxin-7 and caspase-7 and its role on SCA7 disease pathogenesis.
|Young JE,Gouw L,Propp S,Sopher BL,Taylor J,Lin A,Hermel E,Logvinova A,Chen SF,Chen S,Bredesen DE,Truant R,Ptacek LJ,La Spada AR,Ellerby LM||The Journal of biological chemistry (282:30150)||2007|
Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.
PA1-749 was used in immunohistochemistry to study whether spinocerebellar ataxia type 7 neurological features and neurodegeneration are reversible in a conditional murine model
|Furrer SA,Waldherr SM,Mohanachandran MS,Baughn TD,Nguyen KT,Sopher BL,Damian VA,Garden GA,La Spada AR||Human molecular genetics (22:890)||2013|
Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.
PA1-749 was used in immunohistochemistry to investigate the role of ataxin-7 in spinocerebellar ataxia type 7 cerebellar disease
|Furrer SA,Mohanachandran MS,Waldherr SM,Chang C,Damian VA,Sopher BL,Garden GA,La Spada AR||The Journal of neuroscience : the official journal of the Society for Neuroscience (31:16269)||2011|
A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes.
PA1-749 was used in immunohistochemistry to investigate a conditional Drosophila model for spinocerebellar ataxia 7 .
|Latouche M,Lasbleiz C,Martin E,Monnier V,Debeir T,Mouatt-Prigent A,Muriel MP,Morel L,Ruberg M,Brice A,Stevanin G,Tricoire H||The Journal of neuroscience : the official journal of the Society for Neuroscience (27:2483)||2007|
PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.
PA1-749 was used in immunocytochemistry to study the pathogenesis of spinocerebellar ataxia type 7.
|Janer A,Martin E,Muriel MP,Latouche M,Fujigasaki H,Ruberg M,Brice A,Trottier Y,Sittler A||The Journal of cell biology (174:65)||2006|
ADCAII; OPCA3; SCA7; spinocerebellar ataxia type 7 protein
ADCAII; ATXN7; OPCA3; SCA7