The Role of HSP70 and Its Co-chaperones in Protein Misfolding, Aggregation and Disease - The Networking of Chaperones by Co-chaperones - page 256

Table 12.1 (continued)

Protein Disease Main phenotypic

features

Chaperone system Function/putative

function

References

Hsp22/HSPB8 Distal hereditary motor

neuropath (dHMNIIa)

Weakness Small heat shock protein Oligomeric complex

formation with other
sHSP, thus mutations
lead to the formation
of cytotoxic amyloid
protein oligomers and
precipitates

Tang et al. ( 2005b )

Limb muscle atrophy Disruption of cytoskel-

etal networks

Irobi et al. ( 2010 )

Increased downstream
enzymatic activities

HspL27/HSPB3 Distal hereditary motor

neuropath (dHMNIIc)

Weakness Small heat shock protein Oligomeric complex

formation with other
sHSP, thus mutations
lead to the formation
of cytotoxic amyloid
protein oligomers and
precipitates

Kolb et al. ( 2010 )

Limb muscle atrophy Disruption of cytoskel-

etal networks
Increased downstream
enzymatic activities