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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
Amino acid metabolism
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Amino acids are the building blocks of proteins.
And just like how you can make lots of words with a finite alphabet, it’s possible to make lots of proteins with just 20 amino acids!
Each amino acid has nitrogen-containing amine group, and a carboxylic acid - hence the name amino acid!
Each amino acid also has a unique side chain that’s kind of like the amino acid’s fingerprint.
10 of the 20 amino acids are essential, meaning that you obtain them from dietary sources rich in protein, such as meats or tofu.
The other 10 amino acids are non-essential, which means that they can be made in our body, so you don’t have to get them from your diet.
So let’s say you had a nice big bowl of lentils rich in protein.
Protein would get broken down into amino acids, and those amino acids would make their way into various cells to serve as building blocks in protein synthesis.
And the cell has to try to make use of these amino acids, because ammonia which is the nitrogen-containing amine group in amino acids, can become toxic to the cell if it gets freed up and starts to build up in the cell.
Ultimately, to get rid of it, ammonia must first be removed from the amino acid and then sent to the liver where it can get metabolized into a less toxic molecule called urea.
To do that, a group of enzymes called transaminases or aminotransferases transfer that nitrogen containing amino group from amino acids to ketoacids, like alpha-ketoglutarate.
These reactions are called transamination reactions and they’re reversible reactions, meaning that the reaction can go in either direction using the same enzyme.
And generally speaking, transamination reactions requires pyridoxine, or vitamin B6, as a cofactor to help move things along.
So let’s take an example of a transamination reaction with the amino acid alanine in a muscle cell.
First, the enzyme alanine transaminase, or ALT, switches the amino group on alanine with the oxygen group on alpha-ketoglutarate, resulting in a ketoacid called pyruvate, which now has the oxygen group, and the amino acid glutamate, which now has the amino group.
Now pyruvate has two options. First, it can be converted into acetyl-CoA by pyruvate dehydrogenase in the muscle cell, and then the acetyl-CoA can then enter the citric acid cycle.
Second, pyruvate can be converted to lactate by lactate dehydrogenase, and lactate can then travel to the liver.
Because this is a reversible reaction, lactate can be reconverted back to pyruvate in the liver by lactate dehydrogenase as well.
Amino acid metabolism refers to the biochemical pathways that produce, break down, and use amino acids. The body uses amino acids to make proteins, enzymes, hormones, and other important molecules. Protein synthesis and degradation are both essential for maintaining homeostasis in the body.
Amino acid oxidation is a process that helps the body release energy from protein molecules. This process occurs in the liver and muscles and results in the production of ketone bodies, which can be used by cells to generate energy.
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