HSPB1 (heat shock 27kDa protein 1)

Polyclonal antibody produced in rabbits immunized with a synthetic peptide corresponding to a region of Human HSPB1.

Suggested starting concentrations are provided. Optimal dilutions should be determined by end-user. Differences in calculated versus apparent molecular weight may be due to post-translational modifications or protein hydrophobicity. HSPB1. like the other heat shock proteins. is part of a complex system of molecular chaperones in epidermal keratinocytes.

Interacts with TGFB1I1 (By similarity). Associates with alpha- and beta-tubulin and microtubules. Interacts with HSPB8 and HSPBAP1.

Expressed in response to environmental stresses such as heat shock or estrogen stimulation in MCF-7 cells.

Defects in HSPB1 are the cause of Charcot-Marie-Tooth disease type 2F (CMT2F) [MIM:606595]. CMT2F is a form of Charcot-Marie-Tooth disease the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1 and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations normal or slightly reduced nerve conduction velocities and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. CMT2F onset is between 15 and 25 years with muscle weakness and atrophy usually beginning in feet and legs (peroneal distribution). Upper limb involvement occurs later. CMT2F inheritance is autosomal dominant.

Belongs to the small heat shock protein (HSP20) family. Wano. C. . et al. . (2004) Exp. Cell Res. 298 (2). 584-592.