MATR3 Antibody

Code CSB-PA013525GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
Alternative Names
ALS21 antibody; KIAA0723 antibody; Matr3 antibody; MATR3_HUMAN antibody; Matrin-3 antibody; Matrin3 antibody; MPD2 antibody; VCPDM antibody; Vocal cord and pharyngeal weakness with distal myopathy antibody
Raised in
Species Reactivity
Human MATR3
Immunogen Species
Homo sapiens (Human)
Purification Method
Antigen Affinity Purified
It differs from different batches. Please contact us to confirm it.
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
Troubleshooting and FAQs
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs. Plays a role in the regulation of DNA virus-mediated innate immune response by assembling into the HDP-RNP complex, a complex that serves as a platform for IRF3 phosphorylation and subsequent innate immune response activation through the cGAS-STING pathway. May bind to specific miRNA hairpins.
Gene References into Functions
  1. Depletion of SAFB1 reduced FUS's localization to chromatin-bound fraction and splicing activity, suggesting SAFB1 could tether FUS to chromatin compartment thorough N-terminal DNA-binding motif. Moreover, FUS interacts with another nuclear matrix-associated protein, Matrin3. PMID: 27731383
  2. Using a novel RNA pulldown approach that utilized endogenous S1-tagged PINCR (a p53-regulated long noncoding RNA), the authors show that PINCR associates with the enhancer region of these genes by binding to RNA-binding protein Matrin 3 that, in turn, associates with p53. PMID: 28580901
  3. Matrin3 physically interacts with intronic pyrimidine-rich sequences and controls alternative splicing. PMID: 28695676
  4. It may not be a common genetic factor in Chinese amyotrophic lateral sclerosis patients. PMID: 26708275
  5. Its mutations strengthens the role of RNA metabolism in amyotrophic lateral sclerosis etiology. PMID: 26493020
  6. A missense mutation in MATR3 was identified in myopathy patients undergoing a needle electromyography. PMID: 26899464
  7. Our findings indicate that mutations in Matrin 3 that are associated with ALS and myopathy do not dramatically alter the normal localization of the protein or readily induce inclusion formation. PMID: 26528920
  8. Three-dimensional mapping of the lamin A-matrin-3 interface showed that the LMNA truncating mutation Delta303, which lacks the matrin-3 binding domain, was associated with an increased distance between lamin A and matrin-3. PMID: 25948554
  9. Suppressing Matrin 3 powers a heightened and broader ZAP restriction of HIV-1 gene expression. PMID: 26129669
  10. MATR3 mutation is identified to be a possible cause of amyotrophic lateral sclerosis. PMID: 25771394
  11. No mutations were identified, indicating that MATR3 mutations are not a common cause of Amyotrophic lateral sclerosis in Australian familial cases with predominately European ancestry PMID: 25523636
  12. MATR3 gene disruption is associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus. PMID: 25574029
  13. Mutations in MATR3 are rare in French familial ALS and ALS with FTLD patients. PMID: 25158920
  14. This study shows a high risk of abnormal respiratory function with progressive worsening in MATR3 myopathy. PMID: 25677933
  15. Nuclear matrix protein Matrin3 regulates alternative splicing and forms overlapping regulatory networks with PTB. PMID: 25599992
  16. the pY RNA1-s2/Matr3 interaction could play a role in vision PMID: 24558381
  17. Sixteen patients from 6 families with late onset distal myopathy associated with the p.S85C MATR3 mutation were characterized. PMID: 25154462
  18. This study identified mutations in MATR3 in ALS kindreds. also observed MATR3 pathology in ALS-affected spinal cords with and without MATR3 mutations. PMID: 24686783
  19. Data suggest that the cellular level of MATR3, known to be highly regulated, modulates the stability of a group of gene transcripts. PMID: 21858232
  20. Rev needs MATR3 to promote the cytoplasmic accumulation and translation of unspliced RRE-containing mRNA. PMID: 21771348
  21. MATR3 binds viral RNA and is required for the Rev/RRE mediated nuclear export of unspliced HIV-1 RNAs. PMID: 21771346
  22. Matrin 3 binds Rev RNA to stabilize HIV-1 transcripts leading to increased cytoplasmic expression. PMID: 21771347
  23. MATR3 to be a novel ATM target in response to DNA damage. PMID: 20421735
  24. matrin 3 plays a significant role in controlling cell growth and proliferation PMID: 21182838
  25. study of the association of matr3 with chromosome territories and identification of potential interacting proteins PMID: 19562669
  26. Data show that the nuclear matrix protein matrin 3, cytoskeletal motor protein HMP, and the circadian clock protein lark were significantly decreased in fetal Down syndrome brain. PMID: 12469345
  27. These results suggest that the functions of matrin 3 could be regulated by both Ca(2+)-dependent interaction with calmodulin and caspase-mediated cleavage. PMID: 17658460
  28. the spatial proximities among a constellation of functionally related sites that are found within euchromatic regions of the cell nucleus including: HP1gamma, RNA polymerase II, matrin 3, and SAF-A sites PMID: 18618731
  29. nonconservative S85C missense mutation in vocal cord and pharyngeal weakness with distal myopathy PMID: 19344878

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Involvement in disease
Amyotrophic lateral sclerosis 21 (ALS21)
Subcellular Location
Nucleus matrix.
Database Links

HGNC: 6912

OMIM: 164015

KEGG: hsa:9782

STRING: 9606.ENSP00000354346

UniGene: Hs.268939

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