The Relationship Between Protein Misfolding and Human Diseases

The Relationship Between Protein Misfolding and Human Diseases

Research done on diseases caused by protein misfolding: There is little knowledge on the pathological behaviour of diseases in relation to protein misfolding however many recent studies have shown the influence and relation of protein misfolding in diseases. …read more.

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The research data reveals that PAO, pre Amyloid oligomers which is pre soluble is the major toxic entity responsible for pathology in Amyloid plagues and cardiac disorders.

Many of the studies indicated the presence of PAO in many cardiomyocytes of samples in heart failure (Scott and Robbins, 2008). Experiments were conducted in transgenic mice to study the hypothesis of involvement of cardiomyocytes restriction enzyme in heart failure.

The results of these experiments showed that polyglutamine residues of 83 (PQ83) or 19 (PQ19) were expressed by the mice confirming the accumulation of PAO in heart failure (Scott and Robbins, 2008). Further investigation suggests that long PQ lesser than 50 results in toxicity of neurological syndromes and shorter repeats of PQ tend to be benign.

The expression of PQ83 results in accumulation of intracellular PAO which results in formation of aggregates and misfolded proteins causing death of cardiomyocytes leading to heart failure. Additional research proves that there is a presence of increased autophagy and necrosis associated with pathology of PQ83 cardiomyocytes.

These investigations reveal the relationship between protein misfolding and cardiomyocytes death due to increase in intracellular concentration of PAO leading to heart failure (Scott and Robbins, 2008). Many investigations in similar research lines suggest that autophagy as a critical pathway responsible for the deletion of aggregated and misfolded proteins and acts as a critical form for regulation of cell clearance.

The deregulation and malfunctioning of autophagy is revealed as a most important contributing factor for pathogenesis of many cardiac and neurological disorders (Zhu et al., 2007).

Autophagy is revealed as the most essential function in lysosomes and basal cardiomyocytes and up regulation and down regulation could lead to detrimental effects on heart (Zhu et al., 2007).

Therefore all these studies reveal that there is a close relationship between homeostasis of heart and autophagy, misfolding of cardiomyocytes.

3. Protein misfolding in autoimmune disorders: Role of HLA-B27 in spondyloarthropathies The investigation of misfolding caused in the antigen HLA-B27 in relation to spondyloarthropathies is yet to be published. ……………………………………..