|Other Names||Heat shock protein beta-8, HspB8, Alpha-crystallin C chain, E2-induced gene 1 protein, Protein kinase H11, Small stress protein-like protein HSP22, HSPB8, CRYAC, E2IG1, HSP22|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP7139a was selected from the N-term region of human HSPB8. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||CRYAC, E2IG1, HSP22|
|Function||Displays temperature-dependent chaperone activity.|
|Cellular Location||Cytoplasm. Nucleus. Note=Translocates to nuclear foci during heat shock|
|Tissue Location||Predominantly expressed in skeletal muscle and heart.|
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Heat-shock protein beta-8 (HSPB8) displays temperature-dependent chaperone activity. It acts as a Mn(2+)-dependent serine-threonine-specific protein kinase. Defects in HSPB8 are a cause of distal hereditary motor neuropathy type II ; also known as distal spinal muscular atrophy and spinal muscular atrophy of the Charcot-Marie-Tooth type. It is also an autosomal dominant disorder of lower motor neurons characterized by distal muscle weakness.
Irobi, J., et al., Nat. Genet. 36(6):597-601 (2004).Gober, M.D., et al., J. Biol. Chem. 278(39):37600-37609 (2003).Depre, C., et al., Circ. Res. 91(11):1007-1014 (2002).Benndorf, R., et al., J. Biol. Chem. 276(29):26753-26761 (2001).Kappe, G., et al., Biochim. Biophys. Acta 1520(1):1-6 (2001).
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