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Amino acid metabolism
Nitrogen and urea cycle
Citric acid cycle
Electron transport chain and oxidative phosphorylation
Pentose phosphate pathway
Physiological changes during exercise
Fatty acid oxidation
Fatty acid synthesis
Ketone body metabolism
Maple syrup urine disease
Ornithine transcarbamylase deficiency
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hereditary fructose intolerance
Pyruvate dehydrogenase deficiency
Glycogen storage disease type I
Glycogen storage disease type II (NORD)
Glycogen storage disease type III
Glycogen storage disease type IV
Glycogen storage disease type V
Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)
Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)
Fabry disease (NORD)
Gaucher disease (NORD)
Metachromatic leukodystrophy (NORD)
Niemann-Pick disease type C
Niemann-Pick disease types A and B (NORD)
Tay-Sachs disease (NORD)
Disorders of amino acid metabolism: Pathology review
Disorders of carbohydrate metabolism: Pathology review
Disorders of fatty acid metabolism: Pathology review
Dyslipidemias: Pathology review
Glycogen storage disorders: Pathology review
Lysosomal storage disorders: Pathology review
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Your heart is constantly working. Whether you’re swimming or taking a nap, your heart is always on the go.
The main form of energy that keeps our heart cells, and really all of our body cells, going is adenosine triphosphate, or ATP.
In most cells, the main ATP producing factory is the mitochondria, which has an inner and an outer membrane, and it’s along the inner membrane where a process called oxidative phosphorylation occurs.
“Oxidative” refers to oxidation - which is when a molecule donates its electron, and “phosphorylation” which refers to the addition of a phosphate group to adenosine diphosphate, or ADP, to form ATP.
So oxidative phosphorylation is the process of making ATP by donating electrons to complexes embedded within the inner mitochondrial membrane.
These complexes are proteins or lipids coupled with metals like iron and copper that facilitate the movement of electrons.
Together, they form the electron transport chain.
During the electron transport chain, electrons are passed on from complex to complex, and finally to oxygen, creating a proton gradient that will be used to make ATP.
The electron transport chain begins with two key molecules that want to donate their electrons: nicotinamide adenine dinucleotide, or NADH, and flavin adenine dinucleotide, or FADH2, both of which get oxidized in the electron transport chain.
NADH and FADH2 are primarily generated in the citric acid cycle which occurs in the mitochondria, but it can also come directly from glycolysis - which is the breakdown of glucose in the cytoplasm, or fatty acid oxidation, which is the breakdown of fats in the mitochondria.
Enzymes called dehydrogenases help generate the electron-rich NADH and FADH2.
And when those molecules are coming from the cytoplasm they can only enter the mitochondria using a specific shuttle.
When using the malate-aspartate shuttle, electrons enter the electron transport chain as NADH.
When using the glycerol-3-phosphate shuttle, electrons enter electron transport chain as FADH2.
The electron transport chain and oxidative phosphorylation are two biochemical processes that occur in the mitochondria of cells. The electron transport chain is a series of proteins that transfer electrons from donors to acceptors, and oxidative phosphorylation is the process by which the energy released by these electrons is used to generate ATP, which is the cellular currency of energy.
The electron transport chain and oxidative phosphorylation are important for generating energy in all cells, but they are especially important in muscle cells, because muscles use a lot of energy.
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