Small heat shock proteins associated with cerebral amyloid angiopathy of hereditary cerebral hemorrhage with amyloidosis (Dutch type) induce interleukin-6 secretion.

[hereditary cerebral hemorrhage with amyloidosis]

In hereditary cerebral hemorrhage with amyloidosis of the Dutch type (HCHWA-D ), severe cerebral amyloid angiopathy (CAA ) is associated with an inflammatory reaction . Small heat shock proteins (sHsps ) are molecular chaperones and association of HspB 8 with CAA in HCHWA-D has been observed . The aims of this study were to investigate (1 ) if other sHsps are associated with the pathological lesions in HCHWA-D brains , (2 ) if the amyloid-beta protein (A beta ) increases production of sHsps in cultured cerebral cells and (3 ) if sHsps are involved in the cerebral inflammatory processes in both Alzheimer 's disease (AD ) and HCHWA-D . We conclude that Hsp 20 , HspB 8 and HspB 2 are present in CAA in HCHWA-D , and that A beta did not affect cellular sHsps expression in cultured human brain pericytes and astrocytes . In addition , we demonstrated that Hsp 20 , HspB 2 and HspB 8 induced interleukin-6 production in cultured pericytes and astrocytes , which could be antagonized by dexamethasone , whereas other sHsps and A beta were inactive , suggesting that sHsps may be among the key mediators of the local inflammatory response associated with HCHWA-D and AD lesions .

Diseases
Validation

Diseases presenting "amyloidosis of the dutch type" symptom

hereditary cerebral hemorrhage with amyloidosis

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