Short-term succinic acid treatment mitigates cerebellar mitochondrial OXPHOS dysfunction, neurodegeneration and ataxia in a Purkinje-specific spinocerebellar ataxia type 1 (SCA1) mouse model

Succinate, an intermediate in metabolism, signal transduction, ROS, hypoxia, and tumorigenesis

(2016) Laszlo Tretter et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

GPR91: expanding the frontiers of Krebs cycle intermediates

(2016) Matheus de Castro Fonseca et al.   Cell Communication and Signaling

Mitochondrial impairments contribute to Spinocerebellar ataxia type 1 progression and can be ameliorated by the mitochondria-targeted antioxidant MitoQ

(2016) David M. Stucki et al.   FREE RADICAL BIOLOGY AND MEDICINE

Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways

(2016) Melissa Ingram et al.   NEURON

Insight into SUCNR1 (GPR91) structure and function

(2016) Julie Gilissen et al.   PHARMACOLOGY & THERAPEUTICS

Complex I and ATP Content Deficiency in Lymphocytes from Friedreich's Ataxia

(2014) Mohammad Mehdi Heidari et al.   CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES

RAS–MAPK–MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1

(2013) Jeehye Park et al.   NATURE

Succinate: a metabolic signal in inflammation

(2013) Evanna Mills et al.   TRENDS IN CELL BIOLOGY

Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)

(2012) M. Girard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Understanding mitochondrial complex I assembly in health and disease

(2011) Masakazu Mimaki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Changes in circadian rhythms during puberty in Rattus norvegicus: Developmental time course and gonadal dependency

(2011) Megan Hastings Hagenauer et al.   HORMONES AND BEHAVIOR

14-3-3 Binding to Ataxin-1(ATXN1) Regulates Its Dephosphorylation at Ser-776 and Transport to the Nucleus

(2011) Shaojuan Lai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Modeling mitochondrial dysfunctions in the brain: from mice to men

(2011) Megan E. Breuer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Lycopene prevents 3-nitropropionic acid-induced mitochondrial oxidative stress and dysfunctions in nervous system

(2010) Rajat Sandhir et al.   NEUROCHEMISTRY INTERNATIONAL

Mitochondrial Dysfunction in NnaD Mutant Flies and Purkinje Cell Degeneration Mice Reveals a Role for Nna Proteins in Neuronal Bioenergetics

(2010) Lisa Chakrabarti et al.   NEURON

SCA1-like Disease in Mice Expressing Wild-Type Ataxin-1 with a Serine to Aspartic Acid Replacement at Residue 776

(2010) Lisa Duvick et al.   NEURON

Phosphorylation of ATXN1 at Ser776 in the cerebellum

(2009) Nathan D. Jorgensen et al.   JOURNAL OF NEUROCHEMISTRY

A Translational Profiling Approach for the Molecular Characterization of CNS Cell Types

(2008) Myriam Heiman et al.   CELL

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

(2008) Janghoo Lim et al.   NATURE

Analysis of mitochondrial function in situ in permeabilized muscle fibers, tissues and cells

(2008) Andrey V Kuznetsov et al.   Nature Protocols

Methyl succinate antagonises biguanide-induced AMPK-activation and death of pancreatic β-cells through restoration of mitochondrial electron transfer

(2007) S A Hinke et al.   BRITISH JOURNAL OF PHARMACOLOGY