Fueling around with glycogen
The implications of muscle phosphorylase b kinase deficiency
Citation Manager Formats
Make Comment
See Comments
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.
Glycogen is a versatile fuel that provides energy for muscle contraction anaerobically, by substrate-level phosphorylation when glycogen is metabolized to lactic acid, and aerobically, by providing glycogen-derived pyruvate and NADH for oxidative metabolism. Both are required for normal muscle function. Anaerobic glycogenolysis is used to power muscle contractions during maximal exertion since it is able to generate ATP at more than twice the rate of oxidative phosphorylation.1 Glycogen also becomes a critical oxidative substrate in the transition from rest to exercise and in supporting maximal rates of oxidative phosphorylation.1 Glycogen depletion during sustained aerobic activities results in fatigue, and is a rationale for “glycogen loading” before endurance exercise. A survival feature of muscle glycogen is suggested by skeletal muscle lacking the enzyme glucose-6-phosphatase, thus preserving intramuscular glycogen for the energetic needs of fight or flight even in a state of severe caloric restriction,2 whereas liver glycogen is liberated as glucose to support the metabolic requirements of other tissues.
A high demand for limited muscle glycogen requires tight regulation of glycogen phosphorylase, the rate-limiting …
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
For assistance, please contact:
AAN Members (800) 879-1960 or (612) 928-6000 (International)
Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international)
Sign Up
Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here
Purchase
Individual access to articles is available through the Add to Cart option on the article page. Access for 1 day (from the computer you are currently using) is US$ 39.00. Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means. The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use. Distributing copies (electronic or otherwise) of the article is not allowed.
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.
You May Also be Interested in
Hastening the Diagnosis of Amyotrophic Lateral Sclerosis
Dr. Brian Callaghan and Dr. Kellen Quigg
► Watch
Alert Me
Recommended articles
-
Articles
Fat metabolism during exercise in patients with McArdle diseaseM. C. Ørngreen, T. D. Jeppesen, S. Tvede Andersen et al.Neurology, February 23, 2009 -
ARTICLES
Paradoxically enhanced glucose production during exercise in humans with blocked glycolysis caused by muscle phosphofructokinase deficiencyJohn Vissing, Henrik Galbo, Ronald G. Haller et al.Neurology, September 01, 1996 -
Articles
Is muscle glycogenolysis impaired in X-linked phosphorylase b kinase deficiency?M. C. Ørngreen, H. J. Schelhaas, T. D. Jeppesen et al.Neurology, April 09, 2008 -
Clinical Implications of Neuroscience Research
Glycogen metabolismMetabolic coupling between astrocytes and neuronsEduardo E. Benarroch et al.Neurology, March 15, 2010