Gene Symbol: Foxi1
Description: forkhead box I1
Alias: 5830401E05Rik, FREAC6, Fkh10, HFH-3, Hfh3, forkhead box protein I1, HNF-3/forkhead homolog 3, forkhead homolog 10
Species: mouse
Products:     Foxi1

Top Publications

  1. Yang T, Vidarsson H, Rodrigo Blomqvist S, Rosengren S, Enerback S, Smith R. Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4). Am J Hum Genet. 2007;80:1055-63 pubmed
    ..We have identified and characterized a key transcriptional regulatory element in the SLC26A4 promoter that binds FOXI1, a transcriptional activator of SLC26A4. In nine patients with PS or nonsyndromic EVA, a novel c...
  2. Overdier D, Ye H, Peterson R, Clevidence D, Costa R. The winged helix transcriptional activator HFH-3 is expressed in the distal tubules of embryonic and adult mouse kidney. J Biol Chem. 1997;272:13725-30 pubmed
    ..These putative HFH-3 target genes include the Na/K-ATPase, Na/H and anion exchangers, E-cadherin, and mineralocorticoid receptor genes as well as genes for the transcription factors HNF-1, vHNF-1, and HNF-4. ..
  3. Nagalakshmi V, Ren Q, Pugh M, Valerius M, McMahon A, Yu J. Dicer regulates the development of nephrogenic and ureteric compartments in the mammalian kidney. Kidney Int. 2011;79:317-30 pubmed publisher
    ..Furthermore, an understanding of miRNA action may provide new insights into the etiology and pathogenesis of renal cyst-based kidney disease. ..
  4. Jeong H, Jeon U, Koo B, Kim W, Im S, Shin J, et al. Inactivation of Notch signaling in the renal collecting duct causes nephrogenic diabetes insipidus in mice. J Clin Invest. 2009;119:3290-300 pubmed publisher
  5. Hulander M, Kiernan A, Blomqvist S, Carlsson P, Samuelsson E, Johansson B, et al. Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice. Development. 2003;130:2013-25 pubmed
    Mice that lack the winged helix/forkhead gene Foxi1 (also known as Fkh10) are deaf and display shaker/waltzer behavior, an indication of disturbed balance. While Foxi1 is expressed in the entire otic vesicle at E9...
  6. Blomqvist S, Vidarsson H, Fitzgerald S, Johansson B, Ollerstam A, Brown R, et al. Distal renal tubular acidosis in mice that lack the forkhead transcription factor Foxi1. J Clin Invest. 2004;113:1560-70 pubmed
    While macro- and microscopic kidney development appear to proceed normally in mice that lack Foxi1, electron microscopy reveals an altered ultrastructure of cells lining the distal nephron...
  7. Jouret F, Auzanneau C, Debaix H, Wada G, Pretto C, Marbaix E, et al. Ubiquitous and kidney-specific subunits of vacuolar H+-ATPase are differentially expressed during nephrogenesis. J Am Soc Nephrol. 2005;16:3235-46 pubmed
    ..expression of IC-specific a4, B1, G3, and C2 subunits, after the induction of the forkhead transcription factor Foxi1. From E15.5, Foxi1 mRNA was detected in IC, where it co-distributed with B1 in late nephrogenesis...
  8. Blomqvist S, Vidarsson H, Soder O, Enerback S. Epididymal expression of the forkhead transcription factor Foxi1 is required for male fertility. EMBO J. 2006;25:4131-41 pubmed
    ..This depends on active proton secretion into the epididymal lumen. We have identified Foxi1 as an important regulator of gene expression in narrow and clear cells-the major proton secretory cells of ..
  9. Vidarsson H, Westergren R, Heglind M, Blomqvist S, Breton S, Enerback S. The forkhead transcription factor Foxi1 is a master regulator of vacuolar H-ATPase proton pump subunits in the inner ear, kidney and epididymis. PLoS ONE. 2009;4:e4471 pubmed publisher
    ..that expression of four such subunits A1, B1, E2 and a4 all co-localize with the forkhead transcription factor Foxi1 in a subset of epithelial cells at these three locations...

More Information


  1. Kurth I, Hentschke M, Hentschke S, Borgmeyer U, Gal A, Hübner C. The forkhead transcription factor Foxi1 directly activates the AE4 promoter. Biochem J. 2006;393:277-83 pubmed
    ..Mice with a targeted disruption of the gene for the forkhead transcription factor Foxi1 display renal tubular acidosis due to an intercalated cell-differentiation defect...
  2. Clevidence D, Overdier D, Peterson R, Porcella A, Ye H, Paulson K, et al. Members of the HNF-3/forkhead family of transcription factors exhibit distinct cellular expression patterns in lung and regulate the surfactant protein B promoter. Dev Biol. 1994;166:195-209 pubmed
    ..Our results suggest that SPB promoter activity is regulated by HNF-3 alpha and HFH-8 proteins in a cell type-specific manner. ..
  3. Werth M, Schmidt Ott K, Leete T, Qiu A, Hinze C, Viltard M, et al. Transcription factor TFCP2L1 patterns cells in the mouse kidney collecting ducts. elife. 2017;6: pubmed publisher
    ..As a result, Tfcp2l1 regulates the diversification of cell types which is the central characteristic of 'salt and pepper' epithelia and distinguishes the collecting duct from all other nephron segments. ..
  4. Kunisada M, Cui C, Piao Y, Ko M, Schlessinger D. Requirement for Shh and Fox family genes at different stages in sweat gland development. Hum Mol Genet. 2009;18:1769-78 pubmed publisher
    ..During the further development of sweat gland secretory portions, Foxa1 and Foxi1, not at all expressed in hair follicles, were progressively up-regulated in WT but not in Tabby footpads...
  5. Watanabe T, Nakagawa K, Ohata S, Kitagawa D, Nishitai G, Seo J, et al. SEK1/MKK4-mediated SAPK/JNK signaling participates in embryonic hepatoblast proliferation via a pathway different from NF-kappaB-induced anti-apoptosis. Dev Biol. 2002;250:332-47 pubmed
    ..Thus, SEK1 appears to play a crucial role in hepatoblast proliferation and survival in a manner apparently different from NF-kappaB or c-Jun. ..
  6. Hulander M, Wurst W, Carlsson P, Enerback S. The winged helix transcription factor Fkh10 is required for normal development of the inner ear. Nat Genet. 1998;20:374-6 pubmed
    ..Here we report that mice with a targeted disruption of the Fkh10 locus exhibit circling behaviour, poor swimming ability and abnormal reaching response-all common findings in mice ..
  7. Clevidence D, Overdier D, Tao W, Qian X, Pani L, Lai E, et al. Identification of nine tissue-specific transcription factors of the hepatocyte nuclear factor 3/forkhead DNA-binding-domain family. Proc Natl Acad Sci U S A. 1993;90:3948-52 pubmed
    ..The HNF-3/fkh motif therefore comprises a large gene family of transcription factors that play a role in tissue-specific gene regulation and development. ..
  8. Zou D, Silvius D, Rodrigo Blomqvist S, Enerback S, Xu P. Eya1 regulates the growth of otic epithelium and interacts with Pax2 during the development of all sensory areas in the inner ear. Dev Biol. 2006;298:430-41 pubmed
  9. Zaret K. Developmental competence of the gut endoderm: genetic potentiation by GATA and HNF3/fork head proteins. Dev Biol. 1999;209:1-10 pubmed
    ..The mechanistic implications of genetic potentiation and its general significance are discussed. ..
  10. Song R, Preston G, Ichihara A, Yosypiv I. Deletion of the prorenin receptor from the ureteric bud causes renal hypodysplasia. PLoS ONE. 2013;8:e63835 pubmed publisher
    ..had marked kidney hypoplasia, widespread apoptosis of medullary collecting duct cells and decreased expression of Foxi1, AE1 and H(+)-ATPase ?4 mRNA...
  11. Ohyama T, Groves A. Expression of mouse Foxi class genes in early craniofacial development. Dev Dyn. 2004;231:640-6 pubmed
    ..Its expression becomes restricted to the ectoderm and the endoderm of the branchial pouches at E10.5. Foxi1 expression is first detected in the endolymphatic duct in the otic vesicle at E10.5...
  12. Murashima A, Miyagawa S, Ogino Y, Nishida Fukuda H, Araki K, Matsumoto T, et al. Essential roles of androgen signaling in Wolffian duct stabilization and epididymal cell differentiation. Endocrinology. 2011;152:1640-51 pubmed publisher
    ..We also interrogated the possibility of regulation of the p63 gene (Trp63) by AR in vitro and found that p63 is a likely direct target of AR regulation. ..
  13. Raft S, Andrade L, Shao D, Akiyama H, Henkemeyer M, Wu D. Ephrin-B2 governs morphogenesis of endolymphatic sac and duct epithelia in the mouse inner ear. Dev Biol. 2014;390:51-67 pubmed publisher
    ..We propose that developmental dysplasias described here are a gene dose-sensitive cause of the vestibular dysfunction observed in EphB-Efnb2 signaling-deficient mice. ..
  14. Grassmeyer J, Mukherjee M, deRiso J, Hettinger C, Bailey M, Sinha S, et al. Elf5 is a principal cell lineage specific transcription factor in the kidney that contributes to Aqp2 and Avpr2 gene expression. Dev Biol. 2017;424:77-89 pubmed publisher
    ..We have identified Elf5 as an early maker of the principal cell lineage that contributes to the expression of principal cell specific genes. ..