Sir, A recent study by Bannwarth et al.(2014) implicated CHCHD10 as a novel gene for
amyotrophic lateral sclerosis/frontotemporal lobar degeneration (ALS/FTLD), reporting a p …

Mutations in CHCHD10, coding for a mitochondrial intermembrane space protein, are a rare
cause of autosomal dominant amyotrophic lateral sclerosis. Mutation-specific toxic gain of …

The mitochondrion is a highly dynamic organelle that is critical for energy production and
numerous metabolic processes. Drosophila Chchd2, a homolog of the human disease …

Although multiple CHCHD10 mutations are associated with the spectrum of familial and
sporadic frontotemporal dementia–amyotrophic lateral sclerosis (FTD–ALS) diseases …

Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of
heart mitochondrial function activate the mitochondrial integrated stress response (ISR mt), a …

Mutations in coiled-coil-helix-coiled-coil-helix-domain containing 10 (CHCHD10), a
mitochondrial twin CX9C protein whose function is still unknown, cause myopathy, motor …

Objective: To assess the role of CHCHD2 variants in patients with Parkinson disease (PD)
and Lewy body disease (LBD) in Caucasian populations. Methods: All exons of the …

This review considers primary mitochondrial diseases affecting the respiratory chain. As
diseases due to mitochondrial DNA defects defy traditional anatomical classifications, we …

Myopathies are heterogeneous disorders characterized clinically by weakness and
hypotonia, usually in the absence of gross dystrophic changes. Mitochondrial dysfunction is …

Mitochondrial stress triggers a response in the cell's mitochondria and nucleus, but how
these stress responses are coordinated in vivo is poorly understood. Here, we characterize …