ATN1 Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a partial recombinant ATN1.
|Application ||WB, IF, E|
|Calculated MW||125414 Da|
|Other Names||Atrophin-1, Dentatorubral-pallidoluysian atrophy protein, ATN1, D12S755E, DRPLA|
|Target/Specificity||ATN1 (AAH51795, 1 a.a. ~ 110 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.|
|Format||Clear, colorless solution in phosphate buffered saline, pH 7.2 .|
|Storage||Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.|
|Precautions||ATN1 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.|
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Dentatorubral pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder characterized by cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia. The disorder is related to the expansion from 7-23 copies to 49-75 copies of a trinucleotide repeat (CAG/CAA) within this gene. The encoded protein includes a serine repeat and a region of alternating acidic and basic amino acids, as well as the variable glutamine repeat. Alternative splicing results in two transcripts variants that encode the same protein.
Dentatorubral pallidoluysian atrophy in a Turkish family. Yi? U, et al. Turk J Pediatr, 2009 Nov-Dec. PMID 20196398.Screening for premutation in the FMR1 gene in male patients suspected of spinocerebellar ataxia. Rajkiewicz M, et al. Neurol Neurochir Pol, 2008 Nov-Dec. PMID 19235102.Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice. Sato T, et al. Hum Mol Genet, 2009 Feb 15. PMID 19039037.Searching for mutation in the JPH3, ATN1 and TBP genes in Polish patients suspected of Huntington's disease and without mutation in the IT15 gene. Su?ek-Piatkowska A, et al. Neurol Neurochir Pol, 2008 May-Jun. PMID 18651325.Molecular analysis of CAG repeats at five different spinocerebellar ataxia loci: correlation and alternative explanations for disease pathogenesis. Alluri RV, et al. Mol Cells, 2007 Dec 31. PMID 18182848.
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