PMPCAmutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia

Human Frataxin Activates Fe–S Cluster Biosynthesis by Facilitating Sulfur Transfer Chemistry

(2014) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

Spotlight on the July 1 Issue

(2014) R. A. Gross   NEUROLOGY

A Computational Study of the Glycine-Rich Loop of Mitochondrial Processing Peptidase

(2013) Tomáš Kučera et al.   PLoS One

The mitochondrial protein import machinery has multiple connections to the respiratory chain

(2012) Bogusz Kulawiak et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Diagnostic Approach to Childhood-Onset Cerebellar Atrophy

(2012) Almundher Al-Maawali et al.   JOURNAL OF CHILD NEUROLOGY

Recessive Mutations in SPTBN2 Implicate β-III Spectrin in Both Cognitive and Motor Development

(2012) Stefano Lise et al.   PLoS Genetics

CAMOS, a nonprogressive, autosomal recessive, congenital cerebellar ataxia, is caused by a mutant zinc-finger protein, ZNF592

(2010) Elsa Nicolas et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly

(2010) Oleksandr Gakh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Global Analysis of the Mitochondrial N-Proteome Identifies a Processing Peptidase Critical for Protein Stability

(2009) F.-Nora Vögtle et al.   CELL

Reversible oxidation of mitochondrial peroxiredoxin 3 in mouse heart subjected to ischemia and reperfusion

(2009) Vikas Kumar et al.   FEBS LETTERS

The in vivo mitochondrial two-step maturation of human frataxin

(2008) Stéphane Schmucker et al.   HUMAN MOLECULAR GENETICS