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Found Missing
In Short
Function Junction
Plugged In
Filling in the Blanks
Food Pharmacy
Spring 1999
Sausages and cells
--Robert K. Naviaux, M.D., Ph.D.
mitochondriaPhysicians, and most biologists, are taught that mitochondria are monomorphic little sausages in our cells that make adenosine triphosphate (ATP). 

In reality, mitochondria take on many different shapes and subserve a great number of different metabolic functions. Each mitochondrion’s shape is characteristic of the specialized cell in which it resides. 

In the liver, mitochondria are specialized to detoxify ammonia in the urea cycle. Mitochondria are also required for cholesterol metabolism, for estrogen and testosterone synthesis, for neurotransmitter metabolism, and for free radical production and detoxification.

They do all this in addition to breaking down (oxidizing) the fat, protein, and carbohydrates we eat and drink. 

Because mitochondria perform so many different functions in different tissues, there are literally hundreds of different mitochondrial diseases. Even physicians working in highly specialized referral centers who see dozens of cases of mitochondrial disease every year are struck by the great diversity of signs and symptoms. 


Excerpted from “The Spectrum of Mitochondrial Disease”
PICTURE THIS!
 
Ragged red fibers
Succinic dehydrogenase (SDH) stain
Mitochondrial proliferation
 
 
Cytochrome oxidase (COX) stain
Brain pathology in MELAS
Mitochondrial ultrastructure
Related links:
Mitochondrial cytopathy in adults: What we know so far  July 2001 article by Bruce H. Cohen, M.D., and Deborah R. Gold, M.D.  Note: In PDF format.

Mitochondrial Myopathy: An Energy Crisis in the Cell 
August 1999 article from Quest, a magazine published by Muscular Dystrophy Association.

Mitochondrial disease in perspective: Symptoms, diagnosis, and hope for the future 
October 1999 article from Quest magazine.

Mitochondrial Disease Information
UMDF basics on symptoms, diagnosis and treatment.

Mitochondrial Myopathy
Links to mitochondrial news and information on the Muscular Dystrophy Association site.

For a close up view of mitochondria, visit In graphic detail.  For  more about mitochondrial disorders, visit Hot picks and Goal tending
  

Up to one in 4,000 people are affected with a mitochondrial disorder. As more medical professionals learn about them, more individuals are being diagnosed. Spreading the word to the medical community and the public at large is crucial.
                      --UMDF

Mitochondria provide energy that is required for muscles to function properly, so a defect in the mitochondria could severely limit the ability to exercise. 
             --Reuters News
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