Abstract
Amyloidosis are a large group of conformational diseases characterized by abnormal protein folding and assembly which results in the accumulation of insoluble protein aggregates that may accumulate systemically or locally in certain organs or tissue. In local amyloidosis, amyloid deposits are restricted to a particular organ or tissue. Alzheimer’s, Parkinson’s disease, and amyotrophic lateral sclerosis are some examples of neurodegenerative amyloidosis. Local manifestation of protein aggregation in the skin has also been reported. Brain and skin are highly connected at a physiological and pathological level. Recently several studies demonstrated a strong connection between brain and skin in different amyloid diseases. In the present review, we discuss the relevance of the “brain–skin connection” in different neurodegenerative amyloidosis, not only at the pathological level, but also as a strategy for the treatment of these diseases.
Highlights
Amyloidosis is not a single disease, but rather a variety of different pathological processes resulting in the deposition of β-pleated sheet fibrils formed from various proteins by several different pathogenic mechanisms (Husby, 1992)
The three cardinal clinical features of Parkinson’s diseases (PD) are rigidity, resting tremor and bradykinesia, and these occur when approximately 50% of dopaminergic neurons projecting from the substantia nigra pars compacta (SNc) to the striatum are lost (Samii et al, 2004)
There is solid evidence for the existence of a defined “brain– skin connection,” which includes the over-activation of neuropeptides in psoriasis and atopic dermatitis and several dermatologic conditions linked to neuroimmunological stress (Fortune et al, 2002; Biro et al, 2005)
Summary
Amyloidosis is not a single disease, but rather a variety of different pathological processes resulting in the deposition of β-pleated sheet fibrils formed from various proteins by several different pathogenic mechanisms (Husby, 1992). CUTANEOUS AND NEURODEGENERATIVE AMYLOIDOSIS A vast range of human diseases arise from the failure of a specific peptide or protein to adopt or remain in its native functional conformational state. These pathologic conditions collectively are referred to as protein conformational diseases. The largest group of conformational diseases is associated with the conversion of specific peptides or proteins from their soluble functional state to toxic aggregates that are deposited in the tissues (Stefani and Dobson, 2003). Amyloid light chain has been involved in several cases of systemic amyloidosis in the skin (Feito-Rodriguez et al, 2008)
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