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OPA 1 and Its Clinical Implications
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Abstract
Autosomal dominant optic atrophy (ADOA) or Kjer disease (MIM #
165500
) is a primary inherited nonsyndromic optic neuropathy that results in loss of retinal ganglion cells, leading to the clinical appearance of optic atrophy. It is caused by mutations in
optic atrophy 1
(
OPA1
), a dynamin‐related protein of the inner mitochondrial membrane. Recent evidence supports a role for
OPA1
in the regulation of the process of cristae remodelling during apoptosis, by which the complete release of mitochondrial stores of cytochrome
c
is achieved, supporting a role for apoptosis in the pathogenesis of ADOA. Here we review the basic clinical features of ADOA, the biology of
OPA1
and its role in the regulation of mitochondrial shape changes during apoptosis.
Key concepts
Mitochondrial shape changes during apoptosis.
Cristae are the main store of cytochrome
c
.
The complete release of cytochrome
c
is accompanied by the remodelling of the cristae.
The dynamin‐related protein OPA1 regulates the shape of the cristae.
Dis‐oligomerization of OPA1 is a key event in the release of cytochrome
c
during apoptosis.
Title: OPA
1 and Its Clinical Implications
Description:
Abstract
Autosomal dominant optic atrophy (ADOA) or Kjer disease (MIM #
165500
) is a primary inherited nonsyndromic optic neuropathy that results in loss of retinal ganglion cells, leading to the clinical appearance of optic atrophy.
It is caused by mutations in
optic atrophy 1
(
OPA1
), a dynamin‐related protein of the inner mitochondrial membrane.
Recent evidence supports a role for
OPA1
in the regulation of the process of cristae remodelling during apoptosis, by which the complete release of mitochondrial stores of cytochrome
c
is achieved, supporting a role for apoptosis in the pathogenesis of ADOA.
Here we review the basic clinical features of ADOA, the biology of
OPA1
and its role in the regulation of mitochondrial shape changes during apoptosis.
Key concepts
Mitochondrial shape changes during apoptosis.
Cristae are the main store of cytochrome
c
.
The complete release of cytochrome
c
is accompanied by the remodelling of the cristae.
The dynamin‐related protein OPA1 regulates the shape of the cristae.
Dis‐oligomerization of OPA1 is a key event in the release of cytochrome
c
during apoptosis.
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