This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
To the Editor:
In 1996, the gene responsible for Friedreich’s ataxia (FRDA) was shown to encode a small protein, frataxin.1 Decreased frataxin was found to cause a deficiency of mitochondrial iron-sulphur proteins (ISP) in endomyocardial biopsies from FRDA patients.1 The function of this mitochondrial protein is unknown, but recent data support the view that it plays a role in the synthesis of iron-sulphur clusters (ISP) that are subsequently delivered to the various compartments of the cell. The decreased amount of ISP can in turn be the target of a second injury, i.e., an oxidative stress resulting from abnormal mitochondrial iron handling in affected tissues.1 This provided the rationale for an antioxidant treatment by idebenone.2
Altogether, a subsequent large open trial3 and two additional studies recently published in Neurology4,5⇓ showed that idebenone had a significant effect on heart hypertrophy and function in a majority of patients, but not on the neurologic condition in FRDA. The mechanism by which idebenone protects the heart remained to be established. Here, we describe the normalization of mitochondrial ISP activities …
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
