Learn About Optic Atrophy Type 1

What is the definition of Optic Atrophy Type 1?

Optic atrophy type 1 is a condition that often causes slowly worsening vision, usually beginning in childhood. People with optic atrophy type 1 typically experience a narrowing of their field of vision (tunnel vision). Affected individuals gradually lose their sight as their field of vision becomes smaller. Both eyes are usually affected equally, but the severity of the vision loss varies widely, even among affected members of the same family, ranging from nearly normal vision to complete blindness.

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What are the causes of Optic Atrophy Type 1?

Optic atrophy type 1 is caused by mutations in the OPA1 gene. The protein produced from this gene is made in cells and tissues throughout the body. The OPA1 protein is found within mitochondria, which are the energy-producing centers of cells. The protein plays a key role in the organization of the shape and structure of the mitochondria and in controlled cell death (apoptosis). The OPA1 protein is also involved in a process called oxidative phosphorylation, from which cells derive much of their energy. Additionally, the protein plays a role in the maintenance of the DNA within mitochondria, called mitochondrial DNA (mtDNA).

How prevalent is Optic Atrophy Type 1?

Optic atrophy type 1 is estimated to affect 1 in 35,000 people worldwide. This condition is more common in Denmark, where it affects approximately 1 in 10,000 people.

Is Optic Atrophy Type 1 an inherited disorder?

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.

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Who are the sources who wrote this article ?

Published Date: August 01, 2017Published By: National Institutes of Health

What are the Latest Advances for Optic Atrophy Type 1?

There is no recent research available for this condition. Please check back because thousands of new papers are published every week and we strive to find and display the most recent relevant research as soon as it is available.