The meaning of the word mitochondrion (from the Greek mitos, meaning thread, and
chondros, grain) illustrates that the heterogeneity of mitochondrial morphology has been …

Leber hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (DOA) are
the two most common inherited optic neuropathies and they result in significant visual …

Mitochondrial dysfunction is a prominent feature of Alzheimer's disease (AD) neurons. In this
study, we explored the involvement of an abnormal mitochondrial dynamics by investigating …

Mitochondrial fusion requires the coordinated fusion of the outer and inner membranes.
Three large GTPases—OPA1 and the mitofusins Mfn1 and Mfn2—are essential for the …

Aims Mitochondrial fusion and fission are essential processes for preservation of normal
mitochondrial function. We hypothesized that fusion proteins would be decreased in heart …

Since the early days of mitochondrial medicine, it has been clear that optic atrophy is a very
common and sometimes the singular pathological feature in mitochondrial disorders. The …

Discovery of various molecular components regulating dynamics and organization of the
mitochondria in cells, together with novel insights into the role of mitochondrial fusion and …

Optic neuropathy is common in mitochondrial disorders, but poorly characterized in
Friedreich's ataxia (FRDA), a recessive condition caused by lack of the mitochondrial protein …

The OPA1 gene, encoding a dynamin-like mitochondrial GTPase, is involved in autosomal
dominant optic atrophy (ADOA, OMIM# 165500). ADOA, also known as Kjer's optic atrophy …

Nonsyndromic autosomal-recessive optic neuropathies are rare conditions of unknown
genetic and molecular origin. Using an approach of whole-genome homozygosity mapping …