Abstract
Historically, small heat shock proteins (sHSPs) have been extensively studied in the context of being intracellular molecular chaperones. However, recent studies looking at the role of sHSPs in neurological diseases have demonstrated a near universal upregulation of certain sHSPs in damaged and diseased brains. Initially, it was thought that sHSPs are pathological in these disease states because they are found in the areas of damage. However, transgenic overexpression and exogenous administration of sHSPs in various experimental disease paradigms have shown just the contrary – that sHSPs are protective, not pathological. This review examines sHSPs in neurological diseases and highlights the potential for using these neuroprotective sHSPs as novel therapeutics. It first addresses the endogenous expression of sHSPs in a variety of neurological disorders. Although many studies have examined the expression of sHSPs in neurological diseases, there are no review articles summarizing these data. Furthermore, it focuses on recent studies that have investigated the therapeutic potential of sHSPs for neurological diseases. Finally, it will explain what we think is the function of endogenous sHSPs in neurological diseases.
Highlights
OVERVIEW OF SMALL HEAT SHOCK PROTEINS Small heat shock proteins have molecular weights between 12 and 43 kDa, distinguishing them in size from large heat shock proteins (Ganea, 2001; Arrigo et al, 2007)
HSPB2 and HSPB3 are expressed in muscle and heart (Quraishe et al, 2008), a recent study indicates that they have some expression in the brain (Kirbach and Golenhofen, 2011)
Another study did not find upregulation of HSPB1 or HSPB5 in neurons, but rather that localization of these Small heat shock proteins (sHSPs) is specific to glial cells in sporadic corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) and familial FTD with parkinsonism linked to chromosome 17 (FTDP-17) (Dabir et al, 2004)
Summary
B5 Alpha-B crystallin (cryab) Quraishe et al (2008), Kirbach and Golenhofen (2011), Dubin et al (1989). This collection of tauopathies includes frontotemporal lobar degeneration, corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), and familial tauopathy FTD with parkinsonism linked to chromosome 17 (FTDP-17; Kertesz, 2003; Tolnay and Probst, 2003) In many of these conditions, tau pathology is not limited to neurons and instead extends to glial cells such as astrocytes and oligodendrocytes (Komori, 1999). Another study did not find upregulation of HSPB1 or HSPB5 in neurons, but rather that localization of these sHSPs is specific to glial cells in sporadic CBD and PSP and familial FTDP-17 (Dabir et al, 2004). Twenty percent of the familial cases can be attributed to a mutation
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