what are some diseases caused by errors in protien folding, or protein transport?

Answer 1

It is obvious that the structure of a protein and its ability to carry out its correct function are very tightly linked such that small structural defects can lead to a number of protein folding diseases. There are mainly two categories of diseases arising due to protein misfolding.

The first group includes diseases resulting from the lack of a functional protein; the improperly folded proteins are degraded by the cellular machinery. Includes genetic diseases such as cystic fibrosis and sickle cell anemia, which are caused by single residue deletion and mutation respectively, rendering the protein incapable of folding normally.

The second group, amyloidoses consists of diseases arising due to the build up of insoluble protein plaques (amyloids) in the brain or other organs. The protein plaques are found to consist of amyloid fibrils - polymerized cross- beta-sheet structures with the beta-strands arranged perpendicular to the long axis of the fibre. These diseases include prion diseases such as bovine spongiform encephalopathy (BSE) and its human equivalent Creutzfeld-Jakob disease (CJD), and also Alzheimer's disease, Parkinson's disease and type II (non-insulin dependent) diabetes

Answer 2

High Protein Intake Lowers Serotonin Production and
Increases Violence and Aggression
The human body makes use of certain amino acids found in the diet and converts them to specific neurotransmitters. Three primary amino acids in the diet that are important are tyrosine, lecithin (phosphatidylcholine) and tryptophan. The conversion sequences are as follows:

Tyrosine -> Dopa -> Dopamine -> Norepinephrine -> Epinephrine
Lecithin -> Choline -> Choline Acetyltransferase -> Acetylcholine -> Cholinesterase
Tryptophan ->Tryptophan Hydroxylase -> 5-Hydroxytryptophan -> Serotonin

Serotonin is an important substance that inhibits aggression and violence. Most proteins encouraged in the diet contain amino acids other than tryptophan. A high protein meal reduces the plasma ratio of tryptophan to the competing amino acids, and less tryptophan is carried across the blood-brain barrier to the neurons. This fact has socially developed to where those who have a high protein diet (meat, dairy, eggs) are more aggressive. Thus, the high protein diet is encouraged in various sports.

Answer 3

many of the hemoglobinopathies are the result of a point mutation resulting in an abnormal tertiary structure. The protein fails to function normally (transport oxygen) under all conditions.
Sickle cell is the most common, but there are hundreds.

Answer 4

Sickle Cell Anemia - Caused by the change of one amino acid in the hemoglobin (protien) molecule. This change prevents the Hemoglobin molecule to fold the way it should be resulting in Sickle cell anemia.

Answer 5

Amyloidosis