|Reactivity||Human, Mouse, Rat|
|Calculated MW||150 KDa|
|Other Names||Atrophin-1, Dentatorubral-pallidoluysian atrophy protein, ATN1, D12S755E, DRPLA|
|Format||0.01M PBS, pH 7.2, 0.1% Sodium azide, Glycerol 50%|
|Storage||Store at -20 °C.Stable for 12 months from date of receipt|
|Function||Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation (By similarity). Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Asn (polyQ) repeats.|
|Cellular Location||Nucleus. Cytoplasm, perinuclear region. Cell junction. Note=Shuttles between nucleus and cytoplasm. Colocalizes with FAT1 in the perinuclear area, at cell- cell junctions and leading edges of cells (By similarity) Colocalizes with MTG8 in discrete nuclear dots. Proteolytic fragment F1 appears to remain in nucleus. Fragment F2 is exported into the cytoplasm. Fragment F2 from mutant sequences with longer poly-Asn (polyQ) tracts are additionally located to the cytoplasmic membrane and to certain organelles.|
|Tissue Location||Widely expressed in various tissues including heart, lung, kidney, ovary, testis, prostate, placenta, skeletal Low levels in the liver, thymus and leukocytes. In the adult brain, broadly expressed in amygdala, caudate nucleus, corpus callosum, hippocampus, hypothalamus, substantia nigra, subthalamic nucleus, and thalamus. High levels in fetal tissues, especially brain.|
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Transcriptional corepressor. Recruits NR2E1 to repress transcription. Promotes vascular smooth cell (VSMC) migration and orientation (By similarity). Corepressor of MTG8 transcriptional repression. Has some intrinsic repression activity which is independent of the number of poly-Asn (polyQ) repeats.
Nagafuchi S.,et al.Nat. Genet. 8:177-182(1994).
Onodera O.,et al.Am. J. Hum. Genet. 57:1050-1060(1995).
Margolis R.L.,et al.Brain Res. Mol. Brain Res. 36:219-226(1996).
Ansari-Lari M.A.,et al.Genome Res. 7:268-280(1997).
Yanagisawa H.,et al.Hum. Mol. Genet. 5:373-379(1996).
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