Cellular and molecular pathways triggering neurodegeneration in the spinocerebellar ataxias (Q37627670)

21 August 2017

1 reference

title

Cellular and molecular pathways triggering neurodegeneration in the spinocerebellar ataxias (English)

1 reference

21 August 2017

1 reference

Antoni Matilla-Dueñas

1

1 reference

21 August 2017

Ivelisse Sánchez

2

1 reference

21 August 2017

Marc Corral-Juan

3

1 reference

21 August 2017

Antoni Dávalos

4

1 reference

21 August 2017

Ramiro Alvarez

5

1 reference

21 August 2017

Pilar Latorre

6

1 reference

21 August 2017

1 June 2010

1 reference

21 August 2017

1 reference

21 August 2017

9

1 reference

21 August 2017

2

1 reference

21 August 2017

148-166

1 reference

https://scigraph.springernature.com/pub.10.1007/s12311-009-0144-2

21 August 2017

0 references

cites work

Pinning down phosphorylated tau and tauopathies

1 reference

https://api.crossref.org/works/10.1007%2FS12311-009-0144-2

A novel autosomal dominant spinocerebellar ataxia (SCA16) linked to chromosome 8q22.1-24.1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-009-0144-2

Autosomal dominant congenital non-progressive ataxia overlaps with the SCA15 locus

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-009-0144-2

Clinical features and genetic analysis of a new form of spinocerebellar ataxia

21 January 2018

2 references

https://api.crossref.org/works/10.1007%2FS12311-009-0144-2

21 January 2018

12 December 2020

Therapeutic interventions for disease progression in Huntington's disease

1 reference

12 December 2020

Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1.

1 reference

12 December 2020

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

1 reference

12 December 2020

A novel autosomal dominant spinocerebellar ataxia (SCA22) linked to chromosome 1p21-q23.

1 reference

12 December 2020

Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain

1 reference

12 December 2020

Regulation mechanisms and signaling pathways of autophagy

1 reference

12 December 2020

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

1 reference

12 December 2020

Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice

1 reference

12 December 2020

mGluR1 in cerebellar Purkinje cells essential for long-term depression, synapse elimination, and motor coordination

1 reference

12 December 2020

FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons

1 reference

12 December 2020

Mutations in voltage-gated potassium channel KCNC3 cause degenerative and developmental central nervous system phenotypes

1 reference

12 December 2020

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

1 reference

12 December 2020

Clinical and genetic analysis of spinocerebellar ataxia type 11

1 reference

12 December 2020

Insulin-like growth factor I treatment for cerebellar ataxia: Addressing a common pathway in the pathological cascade?

1 reference

12 December 2020

Genetic analysis of SCA 2 and 3 repeat expansions in essential tremor and atypical Parkinsonism.

1 reference

12 December 2020

Expansion of a novel CAG trinucleotide repeat in the 5' region of PPP2R2B is associated with SCA12.

1 reference

12 December 2020

Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders

1 reference

12 December 2020

Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL.

1 reference

12 December 2020

Structural and functional analysis of ataxin-2 and ataxin-3.

1 reference

12 December 2020

Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model

1 reference

12 December 2020

Proteasome function is inhibited by polyglutamine-expanded ataxin-1, the SCA1 gene product

1 reference

12 December 2020

Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7

1 reference

12 December 2020

Deletion at ITPR1 underlies ataxia in mice and spinocerebellar ataxia 15 in humans

1 reference

12 December 2020

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

1 reference

12 December 2020

ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology

1 reference

12 December 2020

Spinocerebellar ataxia type 28: a novel autosomal dominant cerebellar ataxia characterized by slow progression and ophthalmoparesis.

1 reference

12 December 2020

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

12 December 2020

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

1 reference

12 December 2020

Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-x(L)

1 reference

12 December 2020

The highly heterogeneous spinocerebellar ataxias: from genes to targets for therapeutic intervention.

1 reference

12 December 2020

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel

1 reference

12 December 2020

Glutamine repeats and neurodegeneration

1 reference

12 December 2020

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

1 reference

12 December 2020

Protein phosphatase 2A: the Trojan Horse of cellular signaling

1 reference

12 December 2020

Aminopyridines for the treatment of cerebellar and ocular motor disorders.

1 reference

12 December 2020

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.

1 reference

12 December 2020

The Anp32 family of proteins containing leucine-rich repeats.

1 reference

12 December 2020

Haploinsufficiency of AFG3L2, the gene responsible for spinocerebellar ataxia type 28, causes mitochondria-mediated Purkinje cell dark degeneration.

1 reference

12 December 2020

An autosomal dominant cerebellar ataxia linked to chromosome 16q22.1 is associated with a single-nucleotide substitution in the 5' untranslated region of the gene encoding a protein with spectrin repeat and Rho guanine-nucleotide exchange-factor dom

1 reference

12 December 2020

Recent progress in spinocerebellar ataxia type-10 (SCA10).

1 reference

12 December 2020

Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors

1 reference

12 December 2020

Giant SCA8 alleles in nine children whose mother has two moderately large ones

1 reference

12 December 2020

Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration

1 reference

12 December 2020

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

1 reference

12 December 2020

Prospects: histone deacetylase inhibitors

1 reference

12 December 2020

Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities

1 reference

12 December 2020

Missense mutations in the regulatory domain of PKC gamma: a new mechanism for dominant nonepisodic cerebellar ataxia

1 reference

12 December 2020

Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.

1 reference

12 December 2020

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

1 reference

12 December 2020

Large expansion of the ATTCT pentanucleotide repeat in spinocerebellar ataxia type 10.

1 reference

12 December 2020

Autosomal dominant spinocerebellar ataxia with sensory axonal neuropathy (SCA4): clinical description and genetic localization to chromosome 16q22.1

1 reference

12 December 2020

1 reference

12 December 2020

CREB-binding protein sequestration by expanded polyglutamine

1 reference

12 December 2020

The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

1 reference

12 December 2020

Spinocerebellar ataxia type 7 mimicking Kearns-Sayre syndrome: a clinical diagnosis is desirable

1 reference

12 December 2020

Sporadic ataxias in Japan--a population-based epidemiological study.

1 reference

12 December 2020

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

1 reference

12 December 2020

Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice

1 reference

12 December 2020

Evidence for a new spinocerebellar ataxia locus.

1 reference

12 December 2020

Neurogenesis in the adult brain

1 reference

12 December 2020

Spinocerebellar ataxia type 14 caused by a mutation in protein kinase C gamma

1 reference

12 December 2020

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.

1 reference

12 December 2020

REM behavior disorder and excessive daytime somnolence in Machado-Joseph disease (SCA-3).

1 reference

12 December 2020

Bidirectional expression of the SCA8 expansion mutation: one mutation, two genes

1 reference

12 December 2020

A novel protein with RNA-binding motifs interacts with ataxin-2

1 reference

12 December 2020

Protein kinase C: structural and spatial regulation by phosphorylation, cofactors, and macromolecular interactions

1 reference

12 December 2020

Protein aggregation and neurodegenerative disease

1 reference

12 December 2020

Poly-ubiquitin Binding by the Polyglutamine Disease Protein Ataxin-3 Links Its Normal Function to Protein Surveillance Pathways

1 reference

12 December 2020

Insulin-like growth factors in the treatment of neurological disease

1 reference

12 December 2020

Ataxia and paroxysmal dyskinesia in mice lacking axonally transported FGF14.

1 reference

12 December 2020

Mechanisms of cell death in polyglutamine expansion diseases

1 reference

12 December 2020

Chaperoning brain degeneration

1 reference

12 December 2020

Spinocerebellar ataxias type 1, 2 and 3: a study of heart rate variability.

1 reference

12 December 2020

The therapeutic role of creatine in Huntington's disease

1 reference

12 December 2020

Properties of human Cav2.1 channel with a spinocerebellar ataxia type 6 mutation expressed in Purkinje cells.

1 reference

12 December 2020

Cognitive deficits in spinocerebellar ataxia type 1, 2, and 3.

1 reference

12 December 2020

Extrapyramidal motor signs in degenerative ataxias

1 reference

12 December 2020

Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1.

1 reference

12 December 2020

Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

1 reference

12 December 2020

Acetylation: a regulatory modification to rival phosphorylation?

1 reference

12 December 2020

1 reference

12 December 2020

Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation

1 reference

12 December 2020

SCA Functional Index: a useful compound performance measure for spinocerebellar ataxia.

1 reference

12 December 2020

Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death

1 reference

12 December 2020

PKC gamma mutations in spinocerebellar ataxia type 14 affect C1 domain accessibility and kinase activity leading to aberrant MAPK signaling.

1 reference

12 December 2020

RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.

1 reference

12 December 2020

RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice

1 reference

12 December 2020

Functions of Lsm proteins in mRNA degradation and splicing

1 reference

12 December 2020

1 reference

12 December 2020

The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins

1 reference

12 December 2020

Molecular chaperones and the stress of oncogenesis.

1 reference

12 December 2020

Transcriptional alterations and chromatin remodeling in polyglutamine diseases.

1 reference

12 December 2020

Spinocerebellar ataxia type 23: a genetic update

1 reference

12 December 2020

Chinese patients with Machado-Joseph disease presenting with complicated hereditary spastic paraplegia

1 reference

12 December 2020

Six cases of SCA3/MJD patients that mimic hereditary spastic paraplegia in clinic

1 reference

12 December 2020

The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1

1 reference

12 December 2020

RNA gain-of-function in spinocerebellar ataxia type 8.

1 reference

12 December 2020

A new dominantly inherited pure cerebellar ataxia, SCA 30.

1 reference

12 December 2020

Age at onset variance analysis in spinocerebellar ataxias: A study in a Dutch-French cohort

1 reference

12 December 2020

A kindred with cerebellar ataxia and thermoanalgesia.

1 reference

12 December 2020

Analysis of heat shock transcription factor for suppression of polyglutamine toxicity.

1 reference

12 December 2020

A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo

1 reference

12 December 2020

SCA3: neurological features, pathogenesis and animal models

1 reference

12 December 2020

Modulation of neurodegeneration by molecular chaperones

1 reference

12 December 2020

Spinocerebellar ataxia 17 (SCA17) and Huntington's disease-like 4 (HDL4).

1 reference

12 December 2020

A vertebrate RNA-binding protein Fox-1 regulates tissue-specific splicing via the pentanucleotide GCAUG.

1 reference

12 December 2020

Ataxin-7 is a subunit of GCN5 histone acetyltransferase-containing complexes

1 reference

12 December 2020

Scale for the assessment and rating of ataxia: development of a new clinical scale

1 reference

12 December 2020

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

1 reference

12 December 2020

PKC gamma mutant mice exhibit mild deficits in spatial and contextual learning.

1 reference

12 December 2020

Identification and characterization of cholest-4-en-3-one, oxime (TRO19622), a novel drug candidate for amyotrophic lateral sclerosis

1 reference

12 December 2020

A duplication at chromosome 11q12.2-11q12.3 is associated with spinocerebellar ataxia type 20

1 reference

12 December 2020

The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7

1 reference

12 December 2020

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

1 reference

12 December 2020

Enzymological analysis of mutant protein kinase Cgamma causing spinocerebellar ataxia type 14 and dysfunction in Ca2+ homeostasis.

1 reference

12 December 2020

The role of ataxin 10 in the pathogenesis of spinocerebellar ataxia type 10

1 reference

12 December 2020

Spinocerebellar ataxia type 1, 2, and 3 and restless legs syndrome: striatal dopamine D2 receptor status investigated by [11C]raclopride positron emission tomography.

1 reference

12 December 2020

Spinocerebellar ataxia type 26 maps to chromosome 19p13.3 adjacent to SCA6.

1 reference

12 December 2020

Therapeutic effects of cystamine in a murine model of Huntington's disease.

1 reference

12 December 2020

Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions

1 reference

12 December 2020

Caspase activation inhibits proteasome function during apoptosis

1 reference

12 December 2020

Armadillo repeat proteins: beyond the animal kingdom

1 reference

12 December 2020

Clinical and genetic characteristics of non-Asian dentatorubral-pallidoluysian atrophy: A systematic review.

1 reference

12 December 2020

Spinocerebellar ataxia with sensory neuropathy (SCA25)

1 reference

12 December 2020

Molecular pathogenesis of spinocerebellar ataxias.

1 reference

12 December 2020

Spinal cord atrophy in spinocerebellar ataxia type 3 and 6 : impact on clinical disability

1 reference

12 December 2020

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

1 reference

12 December 2020

Dentatorubral-pallidoluysian atrophy: clinical aspects and molecular genetics.

1 reference

12 December 2020

Clinical, psychological, and genetic characteristics of spinocerebellar ataxia type 19 (SCA19).

1 reference

12 December 2020

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

1 reference

12 December 2020

Slowly progressive spinocerebellar ataxia with extrapyramidal signs and mild cognitive impairment (SCA21)

1 reference

12 December 2020

Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration

1 reference

12 December 2020

Calcium channels and channelopathies of the central nervous system

1 reference

12 December 2020

Peripheral nerve involvement in spinocerebellar ataxias

1 reference

12 December 2020

Dimebon improves cognitive function in people with mild to moderate Alzheimer's disease.

1 reference

12 December 2020

Mutations in TTBK2, encoding a kinase implicated in tau phosphorylation, segregate with spinocerebellar ataxia type 11.

1 reference

12 December 2020

Chemical chaperones reduce aggregate formation and cell death caused by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch

1 reference

12 December 2020

Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes

1 reference

12 December 2020

Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1.

1 reference

12 December 2020

Spectrin mutations cause spinocerebellar ataxia type 5.

1 reference

12 December 2020

Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity

1 reference

12 December 2020

Coenzyme Q10 in neurodegenerative diseases

1 reference

12 December 2020

A mutation in the fibroblast growth factor 14 gene is associated with autosomal dominant cerebellar ataxia [corrected]

1 reference

12 December 2020

Spinocerebellar ataxia 2 (SCA2).

1 reference

12 December 2020

Altered dendritic development of cerebellar Purkinje cells in slice cultures from protein kinase Cgamma-deficient mice

1 reference

12 December 2020

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

1 reference

12 December 2020

Histone deacetylase inhibitors reduce polyglutamine toxicity

1 reference

12 December 2020

Mitochondria in neurodegenerative disorders: regulation of the redox state and death signaling leading to neuronal death and survival

1 reference

12 December 2020

Identification of a novel SCA locus ( SCA19) in a Dutch autosomal dominant cerebellar ataxia family on chromosome region 1p21-q21.

1 reference

12 December 2020

Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila.

1 reference

12 December 2020

Effectiveness and safety of treatments for degenerative ataxias: a systematic review.

1 reference

12 December 2020

10.1007/S12311-009-0144-2

Identifiers

DOI

1 reference

21 August 2017

1 reference