Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain

Distinct Pools of Non-Glycolytic Substrates Differentiate Brain Regions and Prime Region-Specific Responses of Mitochondria

(2013) Do Yup Lee et al.   PLoS One

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

(2013) S. C. Johnson et al.   SCIENCE

The lipid peroxidation product 4-hydroxy-2-nonenal: Advances in chemistry and analysis

(2013) Corinne M. Spickett   Redox Biology

A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome

(2012) Z. Assouline et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Fatal breathing dysfunction in a mouse model of Leigh syndrome

(2012) Albert Quintana et al.   JOURNAL OF CLINICAL INVESTIGATION

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

Altered Anesthetic Sensitivity of Mice Lacking Ndufs4, a Subunit of Mitochondrial Complex I

(2012) Albert Quintana et al.   PLoS One

Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity

(2011) M. A. Calvaruso et al.   HUMAN MOLECULAR GENETICS

Isolation of brain mitochondria from neonatal mice

(2011) Xiaoyang Wang et al.   JOURNAL OF NEUROCHEMISTRY

Intrinsic Bioenergetic Properties and Stress Sensitivity of Dopaminergic Synaptosomes

(2011) S. W. Choi et al.   JOURNAL OF NEUROSCIENCE

Clinical progression in Parkinson disease and the neurobiology of axons

(2010) Hsiao-Chun Cheng et al.   ANNALS OF NEUROLOGY

Mitochondrial genetic diseases

(2010) Marni J Falk et al.   CURRENT OPINION IN PEDIATRICS

Coomassie Staining as Loading Control in Western Blot Analysis

(2010) Charlotte Welinder et al.   JOURNAL OF PROTEOME RESEARCH

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Human NARP Mitochondrial Mutation Metabolism Corrected With α-Ketoglutarate/Aspartate

(2009) Gianluca Sgarbi et al.   ARCHIVES OF NEUROLOGY

Bioenergetic analysis of isolated cerebrocortical nerve terminals on a microgram scale: spare respiratory capacity and stochastic mitochondrial failure

(2009) Sung W. Choi et al.   JOURNAL OF NEUROCHEMISTRY

NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement

(2009) E. Leshinsky-Silver et al.   MOLECULAR GENETICS AND METABOLISM

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism