Recombinant Mouse Homeobox protein ARX (Arx)

1. Study characterized the disturbances to cortical interneuron development in mice modeling the two most common ARX polyalanine expansion mutations in human. We found a consistent approximately 40-50% reduction of calbindin-positive interneurons, but not Stt+ or Cr+ interneurons, within the cortex of newborn hemizygous mice for both mutant strains compared to wildtype. PMID: 28627419
2. This report present comprehensive postnatal data for mice modelling the two most common ARX polyalanine expansion mutation and show that the phenotypic outcomes recapitulate the common features observed in patients harbouring ARX polyalanine expansion mutations. PMID: 28602636
3. Study provides further support that the role of Arx in interneuron development is to direct appropriate migration of ventral neuronal precursors into the dorsal cortex and that the loss of Arx results in a failure of interneurons to reach the cortex and thus a deficiency in interneurons. PMID: 27287386
4. ARX positively regulates Wnt/ beta-catenin signaling and the C-terminal domain of ARX interacts with the armadillo repeats in beta-catenin to promote Wnt/beta-catenin signaling. In addition, we found BCL9 and P300 also interact with ARX to modulate Wnt/beta-catenin signaling. PMID: 28103279
5. Arx and Dnmt1 that are sufficient for achieving targeted generation of beta cells from adult pancreatic alpha cells. PMID: 28215845
6. We propose a core pathway of transcription regulators, including Hdac4, involved in chromatin condensation and transcriptional repression, and one of its targets, the transcription factor Twist1, as potential drivers of the ID and infantile spasms in patients with ARX polyalanine expansion mutations PMID: 27798109
7. an expansion of 7 alanines in the first polyalanine tract of both human ARX and mouse Arx altered enteroendocrine differentiation; conclude ARX/Arx is required for the specification of a subset of enteroendocrine cells in both humans and mice PMID: 25171319
8. Results support a model where ARX regulates the expansion of cortical progenitor cells through repression of Cdkn1c PMID: 23968833
9. Our data therefore suggest that strategies aiming at inhibiting the expression of Arx, or its molecular targets/co-factors, may pave new avenues for the treatment of diabetes. PMID: 24204325
10. through interactions with FoxA2, Arx positively regulates Shh expression in the floor plate, and Shh signaling in turn activates Nkx2.2, which suppresses Arx expression. PMID: 24968361
11. ARX in the therian mammal ancestor already played a role in male reproduction before it was recruited to the X chromosome in eutherians. PMID: 24307230
12. Loss of Lmo1 repression may be one of the causes accounting for the phenotypic differences identified between the Arx((GCG)7)and Arx(432-455dup24) mouse models. PMID: 24122442
13. Mice with an expansion mutation in the first polyalanine tract of Arx exhibit impaired alpha-cell specification and maintenance. PMID: 24236044
14. ARX was expressed in the coelomic epithelial cells. PMID: 23840809
15. This study demonistrated that arx is regi=uired for specification of the zona incerta and reticular nucleus of the thalamus in mice. PMID: 24487799
16. nuclear import of Arx via NLS1 contributes to its transcriptional function. PMID: 23771350
17. Pancreatic alpha-cell specific deletion of mouse Arx leads to alpha-cell identity loss. PMID: 23785486
18. Findings indicate that the function of aristaless-related homeobox (ARX) is one of the key modifiers for hyperplasia of islet alpha-like cells in the absence of proglucagon-derived peptides. PMID: 23671715
19. a novel and conserved role of Arx in mammalian endocrine cell development PMID: 22387004
20. these finding demonstrate that, downstream of Neurog3, the specification of a subset of enteroendocrine subtypes relies on both Arx and Pax4, while others depend only on Arx or Pax4. PMID: 22570716
21. study found that although Arx(E)(expanded poly-A tract) functions normally in the dorsal brain, its function in subpallial-derived populations of neurons is compromised; contrasting functions are associated with misregulation of Arx targets through the loss of the ability of Arx(E) to interact with the Arx cofactor Tle PMID: 22108177
22. Nkx2.2 acts by reinforcing the transcriptional networks initiated by Pax4 and Arx in early committed beta- and alpha-cell, respectively. PMID: 21880149
23. Results identified multiple new candidate targets for Arx and should help to better understand the pathophysiological mechanisms of intellectual disability and epilepsy associated with ARX mutations. PMID: 21966449
24. these experiments identify novel genetic interactions between Nkx2.2 and Arx within the endocrine progenitor cells that ensure the correct specification and regulation of endocrine hormone-producing cells PMID: 21856296
25. Isl-1 represents the first known activator of Arx transcription in alpha-cells, here established to be acting through the conserved Re1 and Re2 control domains PMID: 21388963
26. The identification of the lineage determination gene aristaless-related homeobox (Arx), as methylated and repressed in beta cells, and hypomethylated and expressed in alpha cells and Dnmt1-deficient beta cells. PMID: 21497756
27. Ablation of the alpha-cell transcription factor, Arx, resulting in a complete loss of the glucagon-producing pancreatic alpha-cell. PMID: 20592160
28. ARX plays a role in development of forebrain and testes in mice PMID: 12379852
29. Arx is a marker of adult neuronal stem cells and forebrain GABAergic neurons. PMID: 15376319
30. role in regulating the expression of putative instructive signals produced in the olfactory bulb for the proper innervation of olfactory sensory axons PMID: 15677725
31. Arx and Pax4 act as transcriptional repressors that control the expression level of one another, thereby mediating proper endocrine fate allocation in the pancreas. PMID: 15930104
32. Activin A has opposite effects on glucagon and arx gene expression in alpha-cells compared with beta-cells, a finding that may have relevance during pancreatic endocrine lineage specification and physiological function of the adult islets PMID: 16988001
33. In adult mice, the misexpression of Arx, rather than its overexpression, promotes a conversion of beta cells into glucagon- or PP-producing cells in vivo. PMID: 17404619
34. critical functions of Arx in promoting neural migration and regulating basal ganglia differentiation in mice PMID: 17460091
35. Arx acts as a novel positive regulator of embryonic myogenesis by synergizing with Mef2C and MyoD and by establishing an activating loop with Myogenin PMID: 17932502
36. Arx directly repressed three of the identified transcription factors: Lmo1, Ebf3 and Shox2. PMID: 18799476
37. Although Arx is necessary for the Dlx-dependent promotion of interneuron migration, it is not required for the GABAergic cell fate commitment mediated by Dlx factors. PMID: 18923043
38. Arx(-/y);Dlx5/6(CIG) (male) mice exhibit a variety of seizure types beginning in early-life, including seizures that behaviourally and electroencephalographically resembles infantile spasms, and show evolution through development. PMID: 19439424
39. Using an in vitro nuclear import assay, the study shows that import of Arx via nuclear localization sequence2 can be mediated by importin beta1, importin 9, or importin 13, with binding being strongest to importin beta1. PMID: 19494118

Show More

Hide All

KEGG: mmu:11878

STRING: 10090.ENSMUSP00000049039

UniGene: Mm.275547