ATXN3

Gene Symbol	ATXN3
Entrez Gene	4287
Alt Symbol	AT3, ATX3, JOS, MJD, MJD1, SCA3
Species	Human
Gene Type	protein-coding
Description	ataxin 3
Other Description	Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3)|Machado-Joseph disease protein 1|ataxin 3 variant h|ataxin 3 variant m|ataxin 3 variant ref|ataxin-3|josephin|olivopontocerebellar ataxia 3|spinocerebellar ataxia type 3 protein
Swissprots	Q96TC4 Q96TC3 E9PB63 O15286 A7LFZ5 D6RDL9 P54252 Q8N189 O15285 O15284 Q9H3N0
Accessions	ABS29269 ACM69340 BAB55645 BAB55646 CBX53817 CBX53818 EAW81471 EAW81472 EAW81473 EAW81474 EAW81475 P54252 AB050194 BAB18798 AB209309 BAD92546 AK225884 AK307720 AK314919 BC022245 BC033711 AAH33711 BC095402 AAH95402 BU190081 BU663113 DA827537 DQ891282 ABM82208 EU176576 ABW03377 GQ176433 ADD00635 GQ176434 ADD00636 GQ176435 ADD00637 GQ176436 ADD00638 GQ176437 ADD00639 GQ176438 GQ176439 GQ176440 ADD00640 GQ176441 GQ176442 GQ176443 GQ176444 GQ176445 GQ176446 GQ176447 GQ176448 ADD00641 GQ176449 GQ176450 GQ176451 GQ176452 ADD00642 GQ176453 GQ176454 ADD00643 GQ176455 ADD00644 GQ176456 GQ176457 ADD00645 GQ176458 GQ176459 ADD00646 GQ176460 GQ176461 GQ176462 ADD00647 GQ176463 GQ176464 GQ176465 GQ176466 GQ176467 ADD00648 GQ176468 GQ176469 GQ176470 GQ176471 GQ176472 GQ176473 GQ176474 ADD00649 GQ176475 GQ176476 ADD00650 GQ176477 GQ176478 GQ176479 GQ176480 GQ176481 GQ176482 GQ176483 GQ176484 GQ176485 ADD00651 GQ176486 ADD00652 GQ176487 ADD00653 GQ176488 ADD00654 GQ176489 ADD00655 GQ176490 GQ176491 GQ176492 GQ176493 ADD00656 GQ176494 GQ176495 ADD00657 GQ176496 GQ176497 GQ176498 ADD00658 GQ176499 ADD00659 GQ176500 GQ176501 GQ176502 GQ176503 GQ176504 ADD00660 GQ176505 GQ176506 GQ176507 GQ176508 ADD00661 GQ176509 GQ176510 ADD00662 GQ176511 ADD00663 GQ176512 ADD00664 GQ176513 GQ176514 GQ176515 GQ176516 ADD00665 GQ176517 GQ176518 GQ176519 GQ176520 GQ176521 GQ176522 GQ176523 ADD00666 GQ176524 GQ176525 GQ176526 ADD00667 GQ176527 GQ176528 GQ176529 GQ176530 GQ176531 ADD00668 GQ176532 ADD00669 GQ176533 ADD00670 GQ176534 GQ176535 GQ176536 ADD00671 GQ176537 GQ176538 ADD00672 GQ176539 ADD00673 GQ176540 ADD00674 GQ176541 GQ176542 ADD00675 GQ176543 GQ176544 GQ176545 ADD00676 GQ176546 GQ176547 GQ176548 ADD00677 GQ176549 GQ176550 ADD00678 GQ176551 ADD00679 GQ176552 ADD00680 GQ176553 GQ176554 GQ176555 GQ176556 GQ176557 GQ176558 GQ176559 GQ176560 ADD00681 GQ176561 GQ176562 GQ176563 ADD00682 GQ176565 ADD00684 GQ176566 ADD00685 GQ176567 ADD00686 GQ176568 ADD00687 GQ176569 GQ176570 ADD00688 GQ176571 GQ176572 GQ176573 ADD00689 GQ176574 GQ176575 GQ176576 GQ176577 ADD00690 GQ176578 ADD00691 GQ176579 GQ176580 ADD00692 GQ176581 GQ176582 GQ176583 GQ176584 GQ176585 GQ176586 GQ176587 GQ176588 GQ176589 ADD00693 GQ176590 GQ176591 GQ176592 ADD00694 GQ176593 GQ176594 ADD00695 GQ176595 GQ176596 GQ176597 ADD00696 GQ176598 GQ176599 ADD00697 GQ176600 GQ176601 GQ176602 GQ176603 GQ176604 GQ176605 GQ176607 GQ176608 ADD00698 GQ176609 ADD00699 GQ176610 GQ176611 GQ176612 ADD00700 GQ176613 ADD00701 GQ176614 GQ176615 ADD00702 GQ176616 ADD00703 GQ176617 GQ176618 ADD00704 GQ176619 ADD00705 GQ176620 GQ176621 ADD00706 GQ176622 ADD00707 GQ176623 GQ176624 GQ176625 ADD00708 GQ176626 GQ176627 ADD00709 GQ176628 ADD00710 GQ176629 GQ176630 GQ176631 GQ176632 ADD00711 GQ176633 ADD00712 GQ176635 GQ176636 ADD00714 GQ176637 GQ176638 GQ176639 ADD00715 GQ176640 ADD00716 GQ176641 GQ176642 ADD00717 GQ176643 GQ176644 ADD00718 GQ176645 ADD00719 GQ176646 ADD00720 GQ176647 GQ176648 GQ176649 GQ176650 GQ176653 GQ176654 GQ176655 GQ176656 GQ176657 GQ176658 ADD00721 GQ176659 GQ176660 ADD00722 GQ176661 GQ176662 GQ176663 ADD00723 GQ176664 GQ176665 ADD00724 GQ176666 GQ176667 GQ176668 ADD00725 GQ176669 GQ176670 ADD00726 GQ176671 ADD00727 GQ176673 GQ176674 ADD00728 GQ176675 ADD00729 GQ176676 GQ176677 ADD00730 GQ176678 GQ176679 ADD00731 GQ176680 ADD00732 GQ176681 GQ176682 GQ176683 GQ176684 ADD00733 GQ176685 ADD00734 GQ176686 ADD00735 GQ176687 ADD00736 GQ176688 ADD00737 GQ176689 GQ176690 GQ176691 GQ176692 ADD00738 GQ176693 ADD00739 GQ176694 GQ176695 ADD00740 GQ176696 GQ176697 ADD00741 GQ176698 ADD00742 GQ176699 ADD00743 GQ176700 ADD00744 GQ176701 ADD00745 GQ176702 GQ176703 ADD00746 GQ176704 ADD00747 GQ176705 ADD00748 GQ176706 GQ176707 GQ176708 GQ176709 GQ176710 ADD00749 GQ176711 GQ176712 ADD00750 GQ176713 GQ176714 ADD00751 GQ176715 GQ176716 GQ176717 GQ176718 GQ176719 ADD00752 GQ176720 GQ176721 ADD00753 GQ176722 GQ176723 GQ176724 ADD00754 GQ176725 ADD00755 GQ176726 GQ176727 ADD00756 GQ176728 ADD00757 GQ176729 GQ176731 GQ176732 GQ176733 GQ176734 GQ176735 ADD00758 GQ176736 GQ176737 ADD00759 GQ176738 GQ176739 GQ176740 GQ176741 ADD00760 GQ176742 ADD00761 GQ176743 GQ176744 ADD00762 GQ176745 ADD00763 GQ176746 ADD00764 GQ176747 GQ176748 GQ176749 ADD00765 GQ176750 ADD00766 GQ176751 GQ176752 GQ176753 GQ176754 ADD00767 GQ176755 GQ176756 ADD00768 GQ176757 ADD00769 GQ176758 GQ176759 ADD00770 GQ176760 ADD00771 GQ176761 ADD00772 GQ176762 ADD00773 GQ176763 ADD00774 GQ176764 GQ176765 GQ176766 ADD00775 GQ176767 GQ176768 GQ176769 ADD00776 GQ176770 GQ176771 ADD00777 GQ176772 ADD00778 GQ176773 ADD00779 GQ176774 GQ176776 ADD00780 GQ176778 GQ176779 GQ176780 GQ176781 ADD00782 GQ176782 ADD00783 GQ176783 GQ176784 ADD00784 GQ176785 ADD00785 GQ176786 ADD00786 GQ176787 ADD00787 GQ176788 GQ176789 ADD00788 GQ176790 ADD00789 GQ176791 ADD00790 GQ176792 ADD00791 GQ176793 ADD00792 GQ176794 GQ176795 GQ176796 GQ176797 GQ176798 GQ176799 ADD00793 GQ176800 ADD00794 GQ176801 GQ176802 ADD00795 GQ176803 GQ176804 ADD00796 GQ176805 ADD00797 GQ176806 ADD00798 GQ176807 GQ176808 ADD00799 GQ176809 GQ176810 GQ176811 GQ176812 ADD00800 GQ176813 ADD00801 GQ176814 GQ176815 ADD00802 GQ176816 GQ176817 ADD00803 GQ176818 ADD00804 GQ176819 GQ176820 ADD00805 GQ176821 GQ176822 GQ176823 GQ176824 ADD00806 GQ176825 GQ176826 GQ176827 ADD00807 GQ176828 GQ176829 GQ176830 GQ176831 ADD00808 GQ176832 GQ176833 GQ176834 GQ176835 ADD00809 GQ176836 ADD00810 GQ176837 ADD00811 GQ176838 ADD00812 GQ176839 GQ176840 GQ176841 ADD00813 GQ176842 GQ176843 GQ176844 GQ176845 GQ176846 ADD00814 GQ176847 S75313 AAB33571 U64820 AAB63352 U64821 AAB63353 U64822 AAB63354 NM_001127696 NP_001121168 NM_001127697 NP_001121169 NM_001164774 NP_001158246 NM_001164776 NP_001158248 NM_001164777 NP_001158249 NM_001164778 NP_001158250 NM_001164779 NP_001158251 NM_001164780 NP_001158252 NM_001164781 NP_001158253 NM_001164782 NP_001158254 NM_004993 NP_004984 NM_030660 NP_109376 NR_028453 NR_028454 NR_028455 NR_028456 NR_028457 NR_028458 NR_028459 NR_028460 NR_028461 NR_028462 NR_028463 NR_028464 NR_028465 NR_028466 NR_028467 NR_028468 NR_028469 NR_028470 NR_031765 NM_001024631
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. {ECO:0000269|PubMed:12297501, ECO:0000269|PubMed:16118278, ECO:0000269|PubMed:17696782, ECO:0000269|PubMed:23625928}.
Subcellular Location	Nucleus matrix {ECO:0000269|PubMed:9580663}. Note=Predominantly nuclear, but not exclusively, inner nuclear matrix.
Tissue Specificity	Ubiquitous.
Top Pathways	Protein processing in endoplasmic reticulum

• Antibodies
• Assays/Kits
• Proteins/Peptides
• siRNAs/Functional Genomics
• cDNA Clones
• Cell/Tissue/Blood Products

Active human Ataxin 3 full length protein - ab125839 from Abcam Category Protein Source E. coli Species Human

Active human Ataxin 3 full length protein - ab188685 from Abcam Category Protein Prices $232.00 Sizes 50 µg Source E. coli Species Human

Active human Ataxin 3 protein fragment - ab189686 from Abcam Category Protein Prices $519.00 Sizes 250 µg Source E. coli Species Human

Human Ataxin 3 full length protein - ab86706 from Abcam Category Protein Prices $228.00, $866.00 Sizes 50 µg, 250 µg Source E. coli Species Human

Human Ataxin 3 full length protein - ab116441 from Abcam Category Protein Prices $188.00 Sizes 10 µg Source E. coli Species Human

Human Ataxin 3 protein fragment - ab188672 from Abcam Category Protein Prices $461.00 Sizes 250 µl Source E. coli Species Human

Ataxin3-MJD-Antibody-N-term-Blocking-peptide - BP2181a from Abgent, a WuXi AppTec company Category Peptide Prices $80.00 Sizes 100 µg

ATXN3-Antibody-Center-Blocking-Peptide - BP9244c from Abgent, a WuXi AppTec company Category Peptide Prices $80.00 Sizes 100 µg

Recombinant Human Ataxin UIM Domains Agarose, CF - AM-115-250 from R&D Systems Category Protein

Recombinant Human His6-Ataxin-3, CF - E-341-050 from R&D Systems Category Protein Source E. coli-derived, Contains an N-terminal Ser-Tyr-Tyr and 6-His tag

Recombinant Human Ataxin UIM Domains Biotin, CF - UBE-105-250 from R&D Systems Category Protein

Recombinant Human His6-Ataxin UIM Domains, CF - UBE-100-250 from R&D Systems Category Protein

ATXN3 Protein Lysate (APL10548) - APL10548 from Aviva Systems Biology Category Protein Prices $219.00 Sizes 200 µg

ATXN3 Protein Lysate (APL10550) - APL10550 from Aviva Systems Biology Category Protein Prices $219.00 Sizes 200 µg

ATXN3 Protein (OPPA01888) - OPPA01888 from Aviva Systems Biology Category Protein Prices $100.00 Sizes 2 µg Source E. Coli

Antithrombin 3 protein - 30-1046 from Fitzgerald Category Protein Prices $395.00 Sizes 1 mg Species Human

Ataxin 3 protein - 30R-1344 from Fitzgerald Category Protein Prices $255.00 Sizes 50 µg Source E.coli

SERPINC1 protein (His tag) - 80R-3704 from Fitzgerald Category Protein Prices $275.00 Sizes 100 µg Source E.coli

Ataxin-3 (1-370) - AR09225PU-L from Acris Antibodies Category Protein Prices $790.00 Sizes 250 µg Source E. coli Species Human

Ataxin-3 (1-370) - AR09225PU-N from Acris Antibodies Category Protein Prices $340.00 Sizes 50 µg Source E. coli Species Human

Ataxin-3 - H00004287-P01-10 from Acris Antibodies Category Protein Prices $380.00 Sizes 10 µg Species Human

Ataxin-3 - H00004287-P01-25 from Acris Antibodies Category Protein Prices $500.00 Sizes 25 µg Species Human

Ataxin-3 - H00004287-P02 from Acris Antibodies Category Protein Prices $370.00 Sizes 10 µg Species Human

Ataxin-3 - H00004287-P02-10 from Acris Antibodies Category Protein Prices $380.00 Sizes 10 µg Species Human

Ataxin-3 - H00004287-P02-25 from Acris Antibodies Category Protein Prices $500.00 Sizes 25 µg Species Human

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