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Atrophin 1 (ATN1)

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B37; DRPLA; NOD; Dentatorubral Pallidoluysian Atrophy

Atrophin 1 (ATN1)
CCC C225
HGNC 3033
MGI 104725
PubMed 9647693
RGD 61832
UniProt P54259
Wiki ATN1
ATN1 is a protein found in nervous tissue. It is associated with a form of trinucleotide repeat disorder known as "dentatorubral-pallidoluysian atrophy" or "dentatorubropallidoluysian atrophy".
Dentatorubral pallidoluysian atrophy is a rare neurodegenerative disorder characterized by cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia. The disorder is related to the expansion of a trinucleotide repeat 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.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC225Hu01 Recombinant Atrophin 1 (ATN1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC225Hu01 Polyclonal Antibody to Atrophin 1 (ATN1) WB; IHC; ICC; IP.
MAC225Hu21 Monoclonal Antibody to Atrophin 1 (ATN1) WB
Assay Kits SEC225Hu ELISA Kit for Atrophin 1 (ATN1) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Atrophin 1 (ATN1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Atrophin 1 (ATN1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Atrophin 1 (ATN1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Atrophin 1 (ATN1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Atrophin 1 (ATN1) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Atrophin 1 (ATN1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Atrophin 1 (ATN1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Atrophin 1 (ATN1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Atrophin 1 (ATN1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Atrophin 1 (ATN1) ELISA Kit Customized Service Offer
  1. "Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)."Nat. Genet. 8:177-182(1994) [PubMed] [Europe PMC] [Abstract]
  2. "Molecular cloning of a full-length cDNA for dentatorubral-pallidoluysian atrophy and regional expressions of the expanded alleles in the CNS."Am. J. Hum. Genet. 57:1050-1060(1995) [PubMed] [Europe PMC] [Abstract]
  3. "DRPLA gene (atrophin-1) sequence and mRNA expression in human brain."Brain Res. Mol. Brain Res. 36:219-226(1996) [PubMed] [Europe PMC] [Abstract]
  4. "Large-scale sequencing in human chromosome 12p13: experimental and computational gene structure determination."Genome Res. 7:268-280(1997) [PubMed] [Europe PMC] [Abstract]
  5. "A unique origin and multistep process for the generation of expanded DRPLA triplet repeats." Hum. Mol. Genet. 5:373-379(1996) [PubMed] [Europe PMC] [Abstract]
  6. "The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family."Nat. Genet. 7:521-524(1994) [PubMed] [Europe PMC] [Abstract]
  7. "Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis."J. Biol. Chem. 272:29238-29242(1997) [PubMed] [Europe PMC] [Abstract]
  8. "Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins."Mol. Cell. Neurosci. 11:149-160(1998) [PubMed] [Europe PMC] [Abstract]
  9. "Dentatorubral-pallidoluysian atrophy protein interacts through a proline-rich region near polyglutamine with the SH3 domain of an insulin receptor tyrosine kinase substrate."Hum. Mol. Genet. 8:947-957(1999) [PubMed] [Europe PMC] [Abstract]
  10. "Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity."J. Biol. Chem. 274:8730-8736(1999) [PubMed] [Europe PMC] [Abstract]
  11. "Protein binding of a DRPLA family through arginine-glutamic acid dipeptide repeats is enhanced by extended polyglutamine."Hum. Mol. Genet. 9:1433-1442(2000) [PubMed] [Europe PMC] [Abstract]
  12. "Atrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcription."J. Cell Biol. 150:939-948(2000) [PubMed] [Europe PMC] [Abstract]
  13. "Dentatorubral-pallidoluysian atrophy protein is phosphorylated by c-Jun NH2-terminal kinase."Hum. Mol. Genet. 12:1535-1542(2003) [PubMed] [Europe PMC] [Abstract]
  14. "Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity."J. Biol. Chem. 278:13047-13055(2003) [PubMed] [Europe PMC] [Abstract]
  15. "Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."Cell 127:635-648(2006) [PubMed] [Europe PMC] [Abstract]
  16. "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
  17. "Atrophin proteins interact with the Fat1 cadherin and regulate migration and orientation in vascular smooth muscle cells."J. Biol. Chem. 284:6955-6965(2009) [PubMed] [Europe PMC] [Abstract]
  18. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
  19. "Proteolytic processing regulates pathological accumulation in dentatorubral-pallidoluysian atrophy."FEBS J. 277:4873-4887(2010) [PubMed] [Europe PMC] [Abstract]
  20. "Y65C missense mutation in the WW domain of the Golabi-Ito-Hall syndrome protein PQBP1 affects its binding activity and deregulates pre-mRNA splicing."J. Biol. Chem. 285:19391-19401(2010) [PubMed] [Europe PMC] [Abstract]
  21. "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
  22. "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."Sci. Signal. 4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
  23. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]