Gene Symbol: Qk
Description: quaking
Alias: 1110003F05Rik, 1500005P18, QkI, l(17)-1Wis, l17Wis1, protein quaking, QKI-5 protein, QKI-6 protein, QKI-7 protein, QKI-7b protein, lethal, Chr 17, U Wisconsin 1
Species: mouse
Products:     Qk

Top Publications

  1. Hardy R, Loushin C, Friedrich V, Chen Q, Ebersole T, Lazzarini R, et al. Neural cell type-specific expression of QKI proteins is altered in quakingviable mutant mice. J Neurosci. 1996;16:7941-9 pubmed
    b>qkI, a newly cloned gene lying immediately proximal to the deletion in the quakingviable mutation, is transcribed into three messages of 5, 6, and 7 kb...
  2. Zhao L, Mandler M, Yi H, Feng Y. Quaking I controls a unique cytoplasmic pathway that regulates alternative splicing of myelin-associated glycoprotein. Proc Natl Acad Sci U S A. 2010;107:19061-6 pubmed publisher
    ..qk(v)) hypomyelination mutant mouse, diminished expression of isoforms of the selective RNA-binding protein quaking I (QKI) leads to severe dysregulation of MAG splicing...
  3. Larocque D, Pilotte J, Chen T, Cloutier F, Massie B, Pedraza L, et al. Nuclear retention of MBP mRNAs in the quaking viable mice. Neuron. 2002;36:815-29 pubmed
    ..We show that the QKI RNA binding proteins regulate the nuclear export of MBP mRNAs...
  4. Lockhart P, O Farrell C, Farrer M. It's a double knock-out! The quaking mouse is a spontaneous deletion of parkin and parkin co-regulated gene (PACRG). Mov Disord. 2004;19:101-4 pubmed
    ..Homozygous Quaking mice show a complete loss of PRKN and PACRG mRNA and protein. These mice will constitute a useful additional model for studies of the molecular role of parkin and PACRG in neurodegeneration. ..
  5. Ebersole T, Chen Q, Justice M, Artzt K. The quaking gene product necessary in embryogenesis and myelination combines features of RNA binding and signal transduction proteins. Nat Genet. 1996;12:260-5 pubmed
    ..The size and conservation of the quaking gene family implies that the pathway defined by this mutation may have broad relevance for rapid conveyance of extracellular information directly to primary gene transcripts. ..
  6. Larocque D, Galarneau A, Liu H, Scott M, Almazan G, Richard S. Protection of p27(Kip1) mRNA by quaking RNA binding proteins promotes oligodendrocyte differentiation. Nat Neurosci. 2005;8:27-33 pubmed
    ..Here we show that the expression of two QKI isoforms, absent from oligodendrocytes of Qk(v) mice, induces cell cycle arrest of primary rat oligodendrocyte ..
  7. Li Z, Takakura N, Oike Y, Imanaka T, Araki K, Suda T, et al. Defective smooth muscle development in qkI-deficient mice. Dev Growth Differ. 2003;45:449-62 pubmed
    The qkI gene encodes an RNA binding protein which was identified as a candidate for the classical neurologic mutation, qkv...
  8. Chen T, Damaj B, Herrera C, Lasko P, Richard S. Self-association of the single-KH-domain family members Sam68, GRP33, GLD-1, and Qk1: role of the KH domain. Mol Cell Biol. 1997;17:5707-18 pubmed
    ..These observations indicate that the single KH domain found in the Sam68 family, in addition to mediating protein-RNA interactions, mediates protein-protein interactions. ..
  9. Lu Z, Zhang Y, Ku L, Wang H, Ahmadian A, Feng Y. The quakingviable mutation affects qkI mRNA expression specifically in myelin-producing cells of the nervous system. Nucleic Acids Res. 2003;31:4616-24 pubmed
    The genetic lesion in the quakingviable (qk(v)) mutant mice is a deletion 5' to the qkI gene, resulting in severe hypomyelination. qkI produces several QKI protein isoforms via alternative splicing of the C-terminal coding exons...

More Information


  1. Wu J, Reed R, Grabowski P, Artzt K. Function of quaking in myelination: regulation of alternative splicing. Proc Natl Acad Sci U S A. 2002;99:4233-8 pubmed
    ..Here we test the nuclear-localized qk isoform (QKI-5) for its ability to regulate alternative splicing of MAG pre-mRNA in transient coexpression assays...
  2. Wu J, Zhou L, Tonissen K, Tee R, Artzt K. The quaking I-5 protein (QKI-5) has a novel nuclear localization signal and shuttles between the nucleus and the cytoplasm. J Biol Chem. 1999;274:29202-10 pubmed
    The mouse quaking (qk) gene is essential in both myelination and early embryogenesis. Its product, QKI, is an RNA-binding protein belonging to a growing protein family called STAR (signal transduction and activator of RNA)...
  3. Hardy R. QKI expression is regulated during neuron-glial cell fate decisions. J Neurosci Res. 1998;54:46-57 pubmed
    b>QKI proteins are expressed by differentiated glia and have been implicated as regulators of myelination, but are also thought to function during early neural development...
  4. Zhao L, Tian D, Xia M, Macklin W, Feng Y. Rescuing qkV dysmyelination by a single isoform of the selective RNA-binding protein QKI. J Neurosci. 2006;26:11278-86 pubmed
    Alternative splicing of the qkI transcript generates multiple isoforms of the selective RNA-binding protein QKI, which play key roles in controlling the homeostasis of their mRNA targets...
  5. Li Z, Zhang Y, Li D, Feng Y. Destabilization and mislocalization of myelin basic protein mRNAs in quaking dysmyelination lacking the QKI RNA-binding proteins. J Neurosci. 2000;20:4944-53 pubmed
    ..Recently, the genetic lesion of qk(v) has been defined as a deletion 5' to the qkI gene, which results in the severe reduction of the qkI-encoded QKI RNA-binding proteins in myelin-producing cells...
  6. Zhang Y, Lu Z, Ku L, Chen Y, Wang H, Feng Y. Tyrosine phosphorylation of QKI mediates developmental signals to regulate mRNA metabolism. EMBO J. 2003;22:1801-10 pubmed
    The selective RNA-binding protein QKI is essential for myelination in the central nervous system (CNS)...
  7. Noveroske J, Hardy R, Dapper J, Vogel H, Justice M. A new ENU-induced allele of mouse quaking causes severe CNS dysmyelination. Mamm Genome. 2005;16:672-82 pubmed
    ..not evident by sequencing, protein expression studies show that qk(e5)/qk(e5) postnatal oligodendrocytes lack the QKI-6 and QKI-7 isoforms and have reduced QKI-5 levels...
  8. Galarneau A, Richard S. Target RNA motif and target mRNAs of the Quaking STAR protein. Nat Struct Mol Biol. 2005;12:691-8 pubmed
    ..To characterize the RNA binding specificity of the QKI proteins, we selected for RNA species that bound QKI from random pools of RNAs and defined the QKI response element ..
  9. Dapper J, Justice M. Defining the breakpoints of the quaking(viable) mouse mutation reveals a duplication from a Parkin intron. Mov Disord. 2005;20:1369-74 pubmed
    ..The mutation has recently been shown to affect three genes in the region: Quaking (qk), Parkin-coregulated gene (Pacrg), and Parkin...
  10. Pilotte J, Larocque D, Richard S. Nuclear translocation controlled by alternatively spliced isoforms inactivates the QUAKING apoptotic inducer. Genes Dev. 2001;15:845-58 pubmed
    ..The quaking gene encodes at least five alternatively spliced QUAKING (QKI) isoforms that differ in their C-terminal 8--30-amino-acid sequence...
  11. Bo L, Quarles R, Fujita N, Bartoszewicz Z, Sato S, Trapp B. Endocytic depletion of L-MAG from CNS myelin in quaking mice. J Cell Biol. 1995;131:1811-20 pubmed
    ..The loss of L-MAG from quaking periaxonal membranes results from increased endocytosis of L-MAG and possibly a decrease in L-MAG production...
    ..Both mutants offer new opportunities for study of the formation and functions of myelin. ..
  13. Lorenzetti D, Antalffy B, Vogel H, Noveroske J, Armstrong D, Justice M. The neurological mutant quaking(viable) is Parkin deficient. Mamm Genome. 2004;15:210-7 pubmed
    ..This deficiency alters the expression of transcripts from the qkI locus in oligodendrocytes, resulting in improper myelination of the CNS in animals homozygous for the deletion...
  14. Zhao L, Ku L, Chen Y, Xia M, LoPresti P, Feng Y. QKI binds MAP1B mRNA and enhances MAP1B expression during oligodendrocyte development. Mol Biol Cell. 2006;17:4179-86 pubmed
    ..In addition, we found posttranscriptional regulation of MAP1B mRNA by the selective RNA-binding protein QKI in oligodendroglia...
  15. Chen A, Paik J, Zhang H, Shukla S, Mortensen R, Hu J, et al. STAR RNA-binding protein Quaking suppresses cancer via stabilization of specific miRNA. Genes Dev. 2012;26:1459-72 pubmed publisher
    Multidimensional cancer genome analysis and validation has defined Quaking (QKI), a member of the signal transduction and activation of RNA (STAR) family of RNA-binding proteins, as a novel glioblastoma multiforme (GBM) tumor suppressor...
  16. Zearfoss N, Clingman C, Farley B, McCoig L, Ryder S. Quaking regulates Hnrnpa1 expression through its 3' UTR in oligodendrocyte precursor cells. PLoS Genet. 2011;7:e1001269 pubmed publisher
    In mice, Quaking (Qk) is required for myelin formation; in humans, it has been associated with psychiatric disease...
  17. Saccomanno L, Loushin C, Jan E, Punkay E, Artzt K, Goodwin E. The STAR protein QKI-6 is a translational repressor. Proc Natl Acad Sci U S A. 1999;96:12605-10 pubmed
    ..For example, in the mouse, quaking proteins (QKI-5, QKI-6, and QKI-7) are essential for embryogenesis and myelination, whereas a closely related protein in ..
  18. Doukhanine E, Gavino C, Haines J, Almazan G, Richard S. The QKI-6 RNA binding protein regulates actin-interacting protein-1 mRNA stability during oligodendrocyte differentiation. Mol Biol Cell. 2010;21:3029-40 pubmed publisher
    The quaking viable (qk(v)) mice represent an animal model of dysmyelination. The absence of expression of the QKI-6 and QKI-7 cytoplasmic isoforms in oligodendrocytes (OLs) during CNS myelination causes the qk(v) mouse phenotype...
  19. Hardy R. Molecular defects in the dysmyelinating mutant quaking. J Neurosci Res. 1998;51:417-22 pubmed
    ..for the dysmyelinating phenotype had remained elusive, however, until the recent cloning of a candidate gene, qkI (Ebersole et al.: Nature Genet 12:260-265, 1996)...
  20. Chen T, Richard S. Structure-function analysis of Qk1: a lethal point mutation in mouse quaking prevents homodimerization. Mol Cell Biol. 1998;18:4863-71 pubmed
    ..Qk1:EG was consistently more potent at inducing apoptosis than was wild-type Qk1. These results suggest that the mouse quaking lethality (EG) occurs due to the absence of Qk1 self-association mediated by the GSG domain. ..
  21. Lorenzetti D, Bishop C, Justice M. Deletion of the Parkin coregulated gene causes male sterility in the quaking(viable) mouse mutant. Proc Natl Acad Sci U S A. 2004;101:8402-7 pubmed
    Quaking(viable) (qk(v)) is a recessive neurological mouse mutation with severe dysmyelination of the CNS and spermiogenesis failure...
  22. Kondo T, Furuta T, Mitsunaga K, Ebersole T, Shichiri M, Wu J, et al. Genomic organization and expression analysis of the mouse qkI locus. Mamm Genome. 1999;10:662-9 pubmed
    b>qkI, encoding a KH domain-containing RNA binding protein, has been isolated as a candidate gene for the mouse neurological mutation quaking. Here, we describe detailed studies on its genomic structure and expression pattern...
  23. Wilson G, Wang H, Egan G, Robinson P, Delatycki M, O Bryan M, et al. Deletion of the Parkin co-regulated gene causes defects in ependymal ciliary motility and hydrocephalus in the quakingviable mutant mouse. Hum Mol Genet. 2010;19:1593-602 pubmed publisher
    ..1 Mb in the proximal region of chromosome 17. The deletion affects the expression of three genes; quaking (Qk), Parkin-coregulated gene (Pacrg) and parkin (Park2)...
  24. Vernet C, Artzt K. STAR, a gene family involved in signal transduction and activation of RNA. Trends Genet. 1997;13:479-84 pubmed
    ..It is hypothesized that these bifunctional proteins provide a rich source of interesting molecular information about development and define a new cellular pathway that links signal transduction directly to RNA metabolism. ..
  25. Dev A, Nayernia K, Meins M, Adham I, Lacone F, Engel W. Mice deficient for RNA-binding protein brunol1 show reduction of spermatogenesis but are fertile. Mol Reprod Dev. 2007;74:1456-64 pubmed
    ..expression in testes of different mutants with spermatogenesis defects: W/W(V), Tfm/y, Leyl(-/-), olt/olt, and qk/qk. Brunol1 transcript was detectable in Leyl(-/-), olt/olt, and qk/qk mutant but not in W/W(V) and Tfm/y mutants...
  26. Bennett W, Gall A, Southard J, Sidman R. Abnormal spermiogenesis in quaking, a myelin-deficient mutant mouse. Biol Reprod. 1971;5:30-58 pubmed
  27. Ishizuka A, Hasegawa Y, Ishida K, Yanaka K, Nakagawa S. Formation of nuclear bodies by the lncRNA Gomafu-associating proteins Celf3 and SF1. Genes Cells. 2014;19:704-21 pubmed publisher
    ..These observations suggest that Gomafu indirectly modulates the function of the splicing factors SF1 and Celf3 by sequestering these proteins into separate nuclear bodies. ..
  28. Myers K, Liu G, Feng Y, Zheng J. Oligodendroglial defects during quakingviable cerebellar development. Dev Neurobiol. 2016;76:972-82 pubmed publisher
    The selective RNA-binding protein Quaking I (QKI) has previously been implicated in RNA localization and stabilization, alternative splicing, cell proliferation, and differentiation...
  29. Suiko T, Kobayashi K, Aono K, Kawashima T, Inoue K, Ku L, et al. Expression of Quaking RNA-Binding Protein in the Adult and Developing Mouse Retina. PLoS ONE. 2016;11:e0156033 pubmed publisher
    Quaking (QKI), which belongs to the STAR family of KH domain-containing RNA-binding proteins, functions in pre-mRNA splicing, microRNA regulation, and formation of circular RNA...
  30. Adham I, Khulan J, Held T, Schmidt B, Meyer B, Meinhardt A, et al. Fas-associated factor (FAF1) is required for the early cleavage-stages of mouse embryo. Mol Hum Reprod. 2008;14:207-13 pubmed publisher
    ..Thus, our results indicate that the FAF1 gene product is necessary for early embryonic development. ..
  31. Bohnsack B, Lai L, Dolle P, Hirschi K. Signaling hierarchy downstream of retinoic acid that independently regulates vascular remodeling and endothelial cell proliferation. Genes Dev. 2004;18:1345-58 pubmed
  32. Amiguet P, Gardinier M, Zanetta J, Matthieu J. Purification and partial structural and functional characterization of mouse myelin/oligodendrocyte glycoprotein. J Neurochem. 1992;58:1676-82 pubmed
    ..Reduced MOG concentrations were observed in jimpy and quaking dysmyelinating mutant mice, giving further support to its localization in compact myelin of the CNS. ..
  33. Wu Y, Li Z, Yang M, Dai B, Hu F, Yang F, et al. MicroRNA-214 regulates smooth muscle cell differentiation from stem cells by targeting RNA-binding protein QKI. Oncotarget. 2017;8:19866-19878 pubmed publisher
    ..Importantly, miR-214 overexpression in ESCs promoted VSMCs differentiation in vivo. Quaking (QKI) was predicted as one of the major targets of miR-214, which was negatively regulated by miR-214...
  34. Mitrovic N, Le Saux F, Gioanni H, Gioanni Y, Besson M, Maurin Y. Distribution of [3H]clonidine binding sites in the brain of the convulsive mutant quaking mouse: a radioautographic analysis. Brain Res. 1992;578:26-32 pubmed
    ..This comparison emphasizes the role of noradrenaline acting at the level of alpha 2-adrenoceptors in the epileptic syndrome of the quaking mutants. ..
  35. Berger B. [Some ultrastructural aspects of white matter in Quaking mice]. Brain Res. 1971;25:35-53 pubmed
  36. Campagnoni A, Campagnoni C, Bourre J, Jacque C, Baumann N. Cell-free synthesis of myelin basic proteins in normal and dysmyelinating mutant mice. J Neurochem. 1984;42:733-9 pubmed
    Total polyribosomes were isolated from the brains of 16-20 day C57BL/6 mice, four neurological mutants (qk/qk, shi/shi, mld/mld, and jp/Y), and four heterozygote or littermate controls (qk/+, shil/+, mld, and jp littermates) and ..
  37. Lu Z, Ku L, Chen Y, Feng Y. Developmental abnormalities of myelin basic protein expression in fyn knock-out brain reveal a role of Fyn in posttranscriptional regulation. J Biol Chem. 2005;280:389-95 pubmed
    ..Consistent with this idea, Fyn phosphorylates the selective RNA-binding protein QKI, which likely modulates the activity of QKI in binding and stabilizing the MBP mRNA...
  38. Konat G, Trojanowska M, Gantt G, Hogan E. Expression of myelin protein genes in quaking mouse brain. J Neurosci Res. 1988;20:19-22 pubmed
    ..The results indicate a reduced expression of the PLP and BP genes and a developmental delay in the mutant, whereas the genetic expression of MAG is enhanced and appears to be progressively dysregulated...
  39. Ganser A, Kerner A, Brown B, Davisson M, Kirschner D. A survey of neurological mutant mice. II. Lipid composition of myelinated tissue in possible myelin mutants. Dev Neurosci. 1988;10:123-40 pubmed
    ..abstract truncated at 400 words) ..
  40. Koike S, Keino Masu K, Masu M. Deficiency of autotaxin/lysophospholipase D results in head cavity formation in mouse embryos through the LPA receptor-Rho-ROCK pathway. Biochem Biophys Res Commun. 2010;400:66-71 pubmed publisher
    ..These results reveal the signal transduction defects that underlie the abnormalities in Enpp2(-/-) embryos. ..
  41. Li W, Kuchler S, Zaepfel M, Badache A, Thomas D, Vincendon G, et al. Cerebellar soluble lectin and its glycoprotein ligands in the developing brain of control and dysmyelinating mutant mice. Neurochem Int. 1993;22:125-33 pubmed
    ..These results are discussed in view of developmental roles attributed to CSL and its glycoproteins ligands in cell adhesion mechanism during brain ontogenesis and especially myelination. ..
  42. Mitrovic N, Caboche J, Carre J, Besson M, Maurin Y. The quaking mouse: an epileptic mutant with alterations affecting the modulatory mechanisms of the NMDA receptor complex. Brain Res. 1991;566:248-54 pubmed
    ..These alterations might be related to the previously observed anticonvulsant properties of NMDA receptor antagonists in the quaking mouse model of inherited epilepsy...
  43. Cote J, Boisvert F, Boulanger M, Bedford M, Richard S. Sam68 RNA binding protein is an in vivo substrate for protein arginine N-methyltransferase 1. Mol Biol Cell. 2003;14:274-87 pubmed
    ..Other K homology domain RNA binding proteins, including SLM-1, SLM-2, QKI-5, GRP33, and heteronuclear ribonucleoprotein K were also methylated in vivo...
  44. Cox R, Shedlovsky A, Hamvas R, Goldsworthy M, Whittington J, Connelly C, et al. A 1.2-Mb YAC contig spans the quaking region. Genomics. 1994;21:77-84 pubmed
    ..YAC) contig within the region of mouse chromosome 17 between Brachyury (T) and D17Rp17e, and spanning the quaking (qk) region. We describe six new probes distributed across 1...
  45. Hackett B, Brody S, Liang M, Zeitz I, Bruns L, Gitlin J. Primary structure of hepatocyte nuclear factor/forkhead homologue 4 and characterization of gene expression in the developing respiratory and reproductive epithelium. Proc Natl Acad Sci U S A. 1995;92:4249-53 pubmed
  46. Frail D, Braun P. Abnormal expression of the myelin-associated glycoprotein in the central nervous system of dysmyelinating mutant mice. J Neurochem. 1985;45:1071-5 pubmed
    Total cytoplasmic brain RNA was isolated at two different ages from three neurological mutant mice (qk/qk, jp/Y, and shi/shi) and their apparently normal littermates. This RNA was translated in vitro in a rabbit reticulocyte lysate system...
  47. Held T, Barakat A, Mohamed B, Paprotta I, Meinhardt A, Engel W, et al. Heat-shock protein HSPA4 is required for progression of spermatogenesis. Reproduction. 2011;142:133-44 pubmed publisher
    ..These results provide evidence that HSPA4 is required for normal spermatogenesis. ..
  48. Bourre J, Clement M, Gerard D, Chaudiere J. Alterations of cholesterol synthesis precursors (7-dehydrocholesterol, 7-dehydrodesmosterol, desmosterol) in dysmyelinating neurological mutant mouse (quaking, shiverer and trembler) in the PNS and the CNS. Biochim Biophys Acta. 1989;1004:387-90 pubmed
    ..In sciatic nerve, cholesterol is slightly reduced in shiverer, reduced 2-fold in quaking, and dramatically reduced in trembler (10-fold). 7-Dehydrocholesterol is affected in all mutants. ..
  49. Jones S, Johnson K, Yu H, Erway L, Alagramam K, Pollak N, et al. A quantitative survey of gravity receptor function in mutant mouse strains. J Assoc Res Otolaryngol. 2005;6:297-310 pubmed
    ..Homozygotes of Catna2cdf, Grid2ho4J, Wnt1sw, qk, and Mbpshi strains and heterozygotes of Grid2lc had measurable VsEPs but one or more response parameters differed ..
  50. Samorajski T, Friede R, Reimer P. Hypomyelination in the quaking mouse. A model for the analysis of disturbed myelin formation. J Neuropathol Exp Neurol. 1970;29:507-23 pubmed
  51. Kuchler S, Zanetta J, Zaepfel M, Badache A, Sarlieve L, Gumpel M, et al. Endogenous cerebellar soluble lectin and its ligands in central nervous system myelin of quaking and jimpy mutant mice. Dev Neurosci. 1990;12:382-97 pubmed
    ..It is thus postulated that these molecules are essential for ensuring myelin compaction as ligands for the endogenous CSL...
  52. Hayakawa Yano Y, Suyama S, Nogami M, Yugami M, Koya I, Furukawa T, et al. An RNA-binding protein, Qki5, regulates embryonic neural stem cells through pre-mRNA processing in cell adhesion signaling. Genes Dev. 2017;31:1910-1925 pubmed publisher
    ..mRNA sequencing analysis in neural stem cell culture indicates that Qki proteins play supporting roles in the neural stem cell transcriptome and various forms of mRNA processing that may ..
  53. Propst F, Rosenberg M, Oskarsson M, Russell L, Nguyen Huu M, Nadeau J, et al. Genetic analysis and developmental regulation of testis-specific RNA expression of Mos, Abl, actin and Hox-1.4. Oncogene. 1988;2:227-33 pubmed
    ..mouse mutants, dominant spotting (W), sex reversal (Sxr), testicular feminization (Tfm), hypogonadal (hpg), quaking (qk), two t-haplotypes, three X-autosomal translocations, and the YPOS strain, is consistent with its presence in ..
  54. Barbarese E. Spatial distribution of myelin basic protein mRNA and polypeptide in quaking oligodendrocytes in culture. J Neurosci Res. 1991;29:271-81 pubmed
    ..In order to study myelin assembly in the hypomyelinating mutant mouse quaking (qk), cultures of oligodendrocytes were established from affected and control animals...
  55. Larocque D, Richard S. QUAKING KH domain proteins as regulators of glial cell fate and myelination. RNA Biol. 2005;2:37-40 pubmed
    ..The genetic defect in the qk(v) mice prevents the proper expression of alternatively spliced KH-type QKI RNA binding proteins...
  56. Justice M, Bode V. Induction of new mutations in a mouse t-haplotype using ethylnitrosourea mutagenesis. Genet Res. 1986;47:187-92 pubmed
  57. Lakiza O, Frater L, Yoo Y, Villavicencio E, Walterhouse D, Goodwin E, et al. STAR proteins quaking-6 and GLD-1 regulate translation of the homologues GLI1 and tra-1 through a conserved RNA 3'UTR-based mechanism. Dev Biol. 2005;287:98-110 pubmed
    ..We demonstrate that the addition of the mouse QK isoform-6 (QKI-6) protein to a mammalian cell line that lacks QKI-6 can confer regulation on reporter and GLI1 mRNAs in a TGE-..
  58. Nixon R. Increased axonal proteolysis in myelin-deficient mutant mice. Science. 1982;215:999-1001 pubmed
    ..These observations suggest an abnormal axon-glial interaction in mice with primary glial defects and raise the possibility that the functioning of histologically normal axons (neurons) may be altered in dysmyelinating diseases. ..
  59. Billings Gagliardi S, Adcock L, Schwing G, Wolf M. Hypomyelinated mutant mice. II. Myelination in vitro. Brain Res. 1980;200:135-50 pubmed
    ..We have examined the extent to which the culture system reproduces the diseases of three well-known mutants, qk, jpmsd, and jp. Quantitation of myelin profiles per sq...
  60. Mateu L, Luzzati V, Vonasek E, Borgo M, Lachapelle F. Order-disorder phenomena in myelinated nerve sheaths. VI. The effects of quaking, jimpy and shiverer mutations: an X-ray scattering study of mouse sciatic and optic nerves. J Mol Biol. 1996;256:319-29 pubmed
  61. Zorn A, Grow M, Patterson K, Ebersole T, Chen Q, Artzt K, et al. Remarkable sequence conservation of transcripts encoding amphibian and mammalian homologues of quaking, a KH domain RNA-binding protein. Gene. 1997;188:199-206 pubmed
    ..A quaking candidate gene (qkI) that encodes a KH motif protein has recently been identified...
  62. Zhang Y, Feng Y. Distinct molecular mechanisms lead to diminished myelin basic protein and 2',3'-cyclic nucleotide 3'-phosphodiesterase in qk(v) dysmyelination. J Neurochem. 2001;77:165-72 pubmed
    The genetic lesion of quakingviable (qk(v)) causes diminished expression of the QKI RNA-binding protein in myelin producing cells. Consequently, several structural myelin proteins are severely reduced...
  63. Rosenfeld J, Zimmerman A, Friedrich V. Altered brain copper and zinc content in quaking mice. Exp Neurol. 1983;82:55-63 pubmed
    ..Our results indicate abnormal copper content in the quaking mutant. The relationship between copper content and other aspects of the quaking phenotype, including its seizure behavior and myelin deficit, remain to be established. ..
  64. van Mil A, Grundmann S, Goumans M, Lei Z, Oerlemans M, Jaksani S, et al. MicroRNA-214 inhibits angiogenesis by targeting Quaking and reducing angiogenic growth factor release. Cardiovasc Res. 2012;93:655-65 pubmed publisher
    ..The anti-angiogenic effect of miR-214 is mediated through the down-regulation of Quaking and pro-angiogenic growth factor expression. This study presents miR-214 as a potential important target for pro- or anti-angiogenic therapies. ..
  65. Guo W, Jiang T, Lian C, Wang H, Zheng Q, Ma H. QKI deficiency promotes FoxO1 mediated nitrosative stress and endoplasmic reticulum stress contributing to increased vulnerability to ischemic injury in diabetic heart. J Mol Cell Cardiol. 2014;75:131-40 pubmed publisher
    Hearts of diabetic individuals are susceptible to ischemia/reperfusion (I/R) injury. The RNA-binding protein Quaking (QKI) is known to link intracellular signaling to cellular survival and QKI dysregulation may contribute to human ..
  66. Wolf M, Nunnari J, Billings Gagliardi S. Quaking*shiverer double-mutant mice survive for at least 100 days with no CNS myelin. Dev Neurosci. 1999;21:483-90 pubmed
    ..We demonstrate that quaking*shiverer double-mutant mice can survive for at least 100 days without any CNS myelin whatsoever. Therefore, at least for a mouse, absence of CNS myelin is not lethal per se...
  67. Braun P, Horvath E, Edwards A. Two isoforms of myelin-associated glycoprotein accumulate in quaking mice: only the large polypeptide is phosphorylated. Dev Neurosci. 1990;12:286-92 pubmed
    ..In quaking (qk) mutant mouse brain myelin, both MAG polypeptides are evident at all ages examined; the relatively greater abundance ..
  68. Suzuki K, Zagoren J. Quaking mouse: an ultrastructural study of the peripheral nerves. J Neurocytol. 1977;6:71-84 pubmed
    ..N.S. and also during P.N.S. remyelination, we suggest that these features are the results of modified myelination due to a defect in the control mechanisms necessary for normal myelination. ..
  69. Thangaraj M, Furber K, Gan J, Ji S, Sobchishin L, Doucette J, et al. RNA-binding Protein Quaking Stabilizes Sirt2 mRNA during Oligodendroglial Differentiation. J Biol Chem. 2017;292:5166-5182 pubmed publisher
    ..The RNA-binding protein quaking (QKI) plays an important role in myelination by post-transcriptionally regulating the expression of several ..
  70. Le Saux F, Besson M, Maurin Y. Abnormal postnatal ontogeny of the locus coeruleus in the epileptic mutant mouse quaking. Brain Res Dev Brain Res. 2002;136:197-205 pubmed
    ..This developmental abnormality might be a primary determinant of the inherited epilepsy of the quaking mutant mice...