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A few infants lack one of the enzymes of the glycolytic pathway. As a result, they are fed mainly oils and proteins. How these foods bypass the genetic defect (noglycolysis) and allow the baby to live?

2006-09-08 07:14:06 · 6 answers · asked by lil_kim_poster 1 in Science & Mathematics Biology

6 answers

you can generate energy from other molcules besides glucose/carbohydrates, including lipids (oils) and proteins.

however, certain glycolytic intermediates are required as building blocks for important cellular materials. in order to bypass this, i guess the person's cells could undergo gluconeogenesis, essentially running the pathway in reverse. i'm guessing that this aspect would be dependant upon which enzymes were defective.

2006-09-08 09:25:54 · answer #1 · answered by John V 4 · 0 0

A person can get the caloric needs through non-sugar based foods, ie lipids and proteins. Amino Acids can be catabolized into the various intermediates needed in the Kerb cycle. However there are two major problems with that. One is that CoenzymeA(CoA) is needed for continuous Kerb cycle, which is not regenerated efficiently when only lipids/proteins are used. Another is that the brain only uses sugars for energy, thus while the body is intact, the brain becomes malnourished.

The way around that is through Ketogenesis, which is a process where by CoA can be generated, and Keton bodies are made that the brain can use. However at high levels ketogenesis causes the blood to become acidic (Ketoacidosis), and if uncontrolled can be life threatening. This condition usually only affects individuals with acute onset of Type I diabetes.

Now to answer your question: I assume if such a baby is born, then he/she would be taking high doses of CoA supplement, and a tightly monitor blood acidity level.

2006-09-08 11:34:36 · answer #2 · answered by tsubame_z 2 · 0 0

I don't know. Glycosis is the first step in cellular respiration. How do you generate ATP without glycosis? I don't know... maybe some adaptation...

2006-09-08 09:05:42 · answer #3 · answered by cleeps 5 · 0 0

If this were written better, my brain might work. :)

2006-09-08 07:36:09 · answer #4 · answered by johnnylakis 4 · 0 0

wow my brain hurts thanks

2006-09-08 07:16:09 · answer #5 · answered by droid 4 · 0 0

???

2006-09-08 07:16:43 · answer #6 · answered by jb 4 · 0 0

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