Catalogue number: | PA1801 |
Price: | $200.00 |
Reactivities: | Human, Mouse, Rat |
Applications: | Immunocytochemistry, Immunohistochemistry, Immunohistochemistry - frozen, Western Blot |
Size: | 100ug/vial |
Gene: | HSPB1 |
Swiss prot: | P04792 |
Form: | Lyophilized |
Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
Scientific background: | HSPB1(Heat shock 27kDa protein 1), also known as HSP27, is a protein that in humans is encoded by the HSPB1 gene. Stock et al. (2003) used FISH to map the HSP27 gene to 7q11.23. The protein encoded by this gene is induced by environmental stress and developmental changes. The encoded protein is involved in stress resistance and actin organization and translocates from the cytoplasm to the nucleus upon stress induction. Defects in this gene are a cause of Charcot-Marie-Tooth disease type 2F (CMT2F) and distal hereditary motor neuropathy (dHMN). |
References: | 1. Ackerley, S., James, P. A., Kalli, A., French, S., Davies, K. E., Talbot, K. A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes. Hum. Molec. Genet. 15: 347-354, 2006. 2. d'Ydewalle, C., Krishnan, J., Chiheb, D. M., Van Damme, P., Irobi, J., Kozikowski, A. P., Vanden Berghe, P., Timmerman, V., Robberecht, W., Van Den Bosch, L. HDAC6 inhibitors reverse axonal loss in a mouse model of mutant HSPB1-induced Charcot-Marie-Tooth disease. Nature Med. 17: 968-974, 2011. 3. Stock, A. D., Spallone, P. A., Dennis, T. R., Netski, D., Morris, C. A., Mervis, C. B., Hobart, H. H. Heat shock protein 27 gene: chromosomal and molecular location and relationship to Williams syndrome. Am. J. Med. Genet. 120A: 320-325, 2003. |
Additional info: | A synthetic peptide corresponding to a sequence in the middle region of human HSPB1, identical to the related rat and mouse sequences. |