Product References
SCA7 Mouse Cerebellar Pathology Reveals Preferential Downregulation of Key Purkinje Cell-Identity Genes and Shared Disease Signature with SCA1 and SCA2.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Niewiadomska-Cimicka A,Doussau F,Perot JB,Roux MJ,Keime C,Hache A,Piguet F,Novati A,Weber C,Yalcin B,Meziane H,Champy MF,Grandgirard E,Karam A,Messaddeq N,Eisenmann A,Brouillet E,Nguyen HHP,Flament J,Isope P,Trottier Y
PA1-749 was used in Western Blot to reveal a disease signature shared among several SCAs and uncovers potential molecular targets for their treatment.
Wed Jun 02 00:00:00 EDT 2021
DNAzyme Cleavage of CAG Repeat RNA in Polyglutamine Diseases.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
Zhang N,Bewick B,Schultz J,Tiwari A,Krencik R,Zhang A,Adachi K,Xia G,Yun K,Sarkar P,Ashizawa T
PA1-749 was used in Western Blot to develop a DNAzyme capable of destroying expanded CAG repeats present in polyglutamine diseases.
Thu Jul 01 00:00:00 EDT 2021
Respiratory dysfunction in a mouse model of spinocerebellar ataxia type 7.
Disease models & mechanisms
Fusco AF,Pucci LA,Switonski PM,Biswas DD,McCall AL,Kahn AF,Dhindsa JS,Strickland LM,La Spada AR,ElMallah MK
PA1-749 was used in Immunohistochemistry to reveal the importance of phrenic and hypoglossal motor neuron pathology associated with respiratory failure and upper airway dysfunction.
Thu Jul 01 00:00:00 EDT 2021
Metabolic and Organelle Morphology Defects in Mice and Human Patients Define Spinocerebellar Ataxia Type 7 as a Mitochondrial Disease.
Cell reports
Ward JM,Stoyas CA,Switonski PM,Ichou F,Fan W,Collins B,Wall CE,Adanyeguh I,Niu C,Sopher BL,Kinoshita C,Morrison RS,Durr A,Muotri AR,Evans RM,Mochel F,La Spada AR
Published figure using Ataxin 7 polyclonal antibody (Product # PA1-749) in Western Blot
Tue Jan 29 00:00:00 EST 2019
Mutant CAG Repeats Effectively Targeted by RNA Interference in SCA7 Cells.
Genes
Fiszer A,Wroblewska JP,Nowak BM,Krzyzosiak WJ
PA1749 was used in immunocytochemistry to suggest that the selective targeting of a repeat tract in a mutant ATXN7 transcript by RNA interference is a feasible approach to treat spinocerebellar ataxia type 7
Sat Dec 17 00:00:00 EST 2016
Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.
Molecular neurodegeneration
Alves S,Marais T,Biferi MG,Furling D,Marinello M,El Hachimi K,Cartier N,Ruberg M,Stevanin G,Brice A,Barkats M,Sittler A
PA1-749 was used in immunohistochemistry - frozen section and western blot to study recapitulation of SCA7 pathology and promotion of accumulation of the FUS/TLS and MBNL1 RNA-binding proteins by lentiviral vector-mediated overexpression of mutant ataxin
Thu Jul 28 00:00:00 EDT 2016
Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction.
Human molecular genetics
Guyenet SJ,Mookerjee SS,Lin A,Custer SK,Chen SF,Sopher BL,La Spada AR,Ellerby LM
PA1-749 was used in immunohistochemistry and western blot to determine if caspase cleavage contributes to spinocerebellar ataxia type 7 disease pathogenesis using transgenic mice.
Wed Jul 15 00:00:00 EDT 2015
Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.
Molecular therapy : the journal of the American Society of Gene Therapy
Ramachandran PS,Boudreau RL,Schaefer KA,La Spada AR,Davidson BL
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
Mon Sep 01 00:00:00 EDT 2014
Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7.
Molecular therapy : the journal of the American Society of Gene Therapy
Ramachandran PS,Boudreau RL,Schaefer KA,La Spada AR,Davidson BL
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
Mon Sep 01 00:00:00 EDT 2014
RNA interference-based therapy for spinocerebellar ataxia type 7 retinal degeneration.
PloS one
Ramachandran PS,Bhattarai S,Singh P,Boudreau RL,Thompson S,Laspada AR,Drack AV,Davidson BL
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
Fri Jan 09 00:00:00 EST 2015
Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.
Brain : a journal of neurology
Chort A,Alves S,Marinello M,Dufresnois B,Dornbierer JG,Tesson C,Latouche M,Baker DP,Barkats M,El Hachimi KH,Ruberg M,Janer A,Stevanin G,Brice A,Sittler A
PA1-749 was used in Western Blotting and Immunohistochemistry to show that interferon beta induces PML-dependent clearance of ataxin-7, and improves motor function.
Sat Jun 01 00:00:00 EDT 2013
Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.
Brain : a journal of neurology
Chort A,Alves S,Marinello M,Dufresnois B,Dornbierer JG,Tesson C,Latouche M,Baker DP,Barkats M,El Hachimi KH,Ruberg M,Janer A,Stevanin G,Brice A,Sittler A
PA1-749 was used in Western Blotting and Immunohistochemistry to show that interferon beta induces PML-dependent clearance of ataxin-7, and improves motor function.
Sat Jun 01 00:00:00 EDT 2013
Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.
Human molecular genetics
Furrer SA,Waldherr SM,Mohanachandran MS,Baughn TD,Nguyen KT,Sopher BL,Damian VA,Garden GA,La Spada AR
PA1-749 was used in immunohistochemistry to study whether spinocerebellar ataxia type 7 neurological features and neurodegeneration are reversible in a conditional murine model
Fri Mar 01 00:00:00 EST 2013
Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model.
Molecular neurodegeneration
Duncan CE,An MC,Papanikolaou T,Rugani C,Vitelli C,Ellerby LM
Published figure using Ataxin 7 polyclonal antibody (Product # PA1-749) in Western Blot
Sun Oct 27 00:00:00 EDT 2013
Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model.
Molecular neurodegeneration
Duncan CE,An MC,Papanikolaou T,Rugani C,Vitelli C,Ellerby LM
Published figure using Ataxin 7 polyclonal antibody (Product # PA1-749) in Western Blot
Sun Oct 27 00:00:00 EDT 2013
Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model.
Molecular neurodegeneration
Duncan CE,An MC,Papanikolaou T,Rugani C,Vitelli C,Ellerby LM
Published figure using Ataxin 7 polyclonal antibody (Product # PA1-749) in Western Blot
Sun Oct 27 00:00:00 EDT 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.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Furrer SA,Mohanachandran MS,Waldherr SM,Chang C,Damian VA,Sopher BL,Garden GA,La Spada AR
PA1-749 was used in immunohistochemistry to investigate the role of ataxin-7 in spinocerebellar ataxia type 7 cerebellar disease
Wed Nov 09 00:00:00 EST 2011
Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Mookerjee S,Papanikolaou T,Guyenet SJ,Sampath V,Lin A,Vitelli C,DeGiacomo F,Sopher BL,Chen SF,La Spada AR,Ellerby LM
PA1-749 was used in western blot and immunoprecipitation to study the effect of ataxin-7 post-translational modification on its autophagy-mediated proteolytic processing.
Wed Dec 02 00:00:00 EST 2009
Posttranslational modification of ataxin-7 at lysine 257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Mookerjee S,Papanikolaou T,Guyenet SJ,Sampath V,Lin A,Vitelli C,DeGiacomo F,Sopher BL,Chen SF,La Spada AR,Ellerby LM
PA1-749 was used in western blot and immunoprecipitation to study the effect of ataxin-7 post-translational modification on its autophagy-mediated proteolytic processing.
Wed Dec 02 00:00:00 EST 2009
A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes.
The Journal of neuroscience : the official journal of the Society for Neuroscience
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
PA1-749 was used in immunohistochemistry to investigate a conditional Drosophila model for spinocerebellar ataxia 7 .
Wed Mar 07 00:00:00 EST 2007
Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation.
The Journal of biological chemistry
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
PA1-749 was used in western blot to study the relationship between ataxin-7 and caspase-7 and its role on SCA7 disease pathogenesis.
Fri Oct 12 00:00:00 EDT 2007
PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.
The Journal of cell biology
Janer A,Martin E,Muriel MP,Latouche M,Fujigasaki H,Ruberg M,Brice A,Trottier Y,Sittler A
PA1-749 was used in immunocytochemistry to study the pathogenesis of spinocerebellar ataxia type 7.
Mon Jul 03 00:00:00 EDT 2006
PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.
The Journal of cell biology
Janer A,Martin E,Muriel MP,Latouche M,Fujigasaki H,Ruberg M,Brice A,Trottier Y,Sittler A
PA1-749 was used in immunocytochemistry to study the pathogenesis of spinocerebellar ataxia type 7.
Mon Jul 03 00:00:00 EDT 2006
PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.
The Journal of cell biology
Janer A,Martin E,Muriel MP,Latouche M,Fujigasaki H,Ruberg M,Brice A,Trottier Y,Sittler A
PA1-749 was used in immunocytochemistry to study the pathogenesis of spinocerebellar ataxia type 7.
Mon Jul 03 00:00:00 EDT 2006