|Other Names||Proteasome assembly chaperone 1, PAC-1, Chromosome 21 leucine-rich protein, C21-LRP, Down syndrome critical region protein 2, PSMG1, C21LRP, DSCR2, PAC1|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP6318a was selected from the N-term region of human DSCR2. 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||C21LRP, DSCR2, PAC1|
|Function||Chaperone protein which promotes assembly of the 20S proteasome as part of a heterodimer with PSMG2. The PSMG1-PSMG2 heterodimer binds to the PSMA5 and PSMA7 proteasome subunits, promotes assembly of the proteasome alpha subunits into the heteroheptameric alpha ring and prevents alpha ring dimerization.|
|Cellular Location||Cytoplasm. Endoplasmic reticulum.|
|Tissue Location||In the adult, detected in brain, colon, leukocytes, breast and testis. Widely expressed in the fetus. Also expressed in a variety of proliferating cell lines|
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Down syndrome, the most common birth defect, is characterized by distinct facial and physical features and mental retardation and is caused by trisomy 21. The chromosome region located between DNA marker D21S55 and MX1 in 21q22.3 is referred to as the Down syndrome critical region 2 and is associated with certain features of the syndrome. By searching an EST database for genes between markers D21S343 and D21S268 in Down syndrome critical region 2, Vidal-Taboada et al. (1998) identified DSCR2. Northern blot analysis detects high expression of a 1.3-kb DSCR2 transcript in testis and in the Jurkat leukemia cell line. RT-PCR analysis detects DSCR2 mRNA in brain, colon, leukocytes, breast, and testis, as well as in all fetal tissues tested.
Vesa, J., et al., Biochem. Biophys. Res. Commun. 328(1):235-242 (2005).Possik, P.A., et al., Biochem. Biophys. Res. Commun. 48(3):267-272 (2004).Vidal-Taboada, J.M., et al., Biochem. Biophys. Res. Commun. 250(3):547-554 (1998).
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