Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3
Recombinant human Ataxin-3 protein (1-288AA)
ELISA;Not yet tested in other applications.
Background / Function
Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription.
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.
Caprylic Acid Ammonium Sulfate Precipitation purified
Literature”Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain.”Mao Y., Senic-Matuglia F., Di Fiore P.P., Polo S., Hodsdon M.E., De Camilli P.Proc. Natl. Acad. Sci. U.S.A. 102:12700-12705(2005). ”The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognition.”Nicastro G., Menon R.P., Masino L., Knowles P.P., McDonald N.Q., Pastore A.Proc. Natl. Acad. Sci. U.S.A. 102:10493-10498(2005). ”Structural modeling of ataxin-3 reveals distant homology to adaptins.” Albrecht M., Hoffmann D., Evert B.O., Schmitt I., Wuellner U., Lengauer T. Proteins 50:355-370(2003).