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Gene Review

Llgl1  -  lethal giant larvae homolog 1 (Drosophila)

Mus musculus

Synonyms: AI325176, LLGL, Lethal(2) giant larvae protein homolog 1, Lgl1, Llglh, ...
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High impact information on Llgl1


Biological context of Llgl1

  • We have isolated and characterized the human homologue of the murine Llglh gene, which was originally isolated as a homologue of a Drosophila tumor suppressor gene 1(2)gl (lethal(2) giant larvae) [3].
  • In our FISH analysis of metaphase chromosomes of four SMS patients, a probe representing LLGL failed in each case to hybridize to one of the two chromosome 17 homologues, indicating that this gene may play a role in the pathogenesis of SMS [3].
  • Phosphorylation within a highly conserved region of Mlgl is required to restrict the protein to the lateral domain, because a recombinant phospho-mutant is distributed in a nonpolar manner [2].
  • Consistent with a role in cell polarity, Mlgl redistributes from a cytoplasmic localization to the lateral membrane after contact-naive MDCK cells make cell-cell contacts and establish a polarized phenotype [2].
  • Fliih, the murine homologue of the Drosophila melanogaster flightless I gene: nucleotide sequence, chromosomal mapping and overlap with Llglh [4].

Associations of Llgl1 with chemical compounds

  • Interestingly, mutant forms of Mgl-1 at the conserved glycine at position 450 and aspartic acid at position 453 in the most conserved WD-40 repeat motif were not able to complement, indicating that these amino acids are critical for regulating salt tolerance and temperature sensitivity in yeast [5].

Other interactions of Llgl1

  • In the mouse, Llglh is thought to play an important role during brain development as a regulatory target of Hoxc8 [3].
  • Fliih lies adjacent to Llglh, the mouse homologue of the D. melanogaster tumor suppressor gene lethal(2) giant larvae [4].


  1. A polarity complex of mPar-6 and atypical PKC binds, phosphorylates and regulates mammalian Lgl. Plant, P.J., Fawcett, J.P., Lin, D.C., Holdorf, A.D., Binns, K., Kulkarni, S., Pawson, T. Nat. Cell Biol. (2003) [Pubmed]
  2. Mammalian homolog of Drosophila tumor suppressor lethal (2) giant larvae interacts with basolateral exocytic machinery in Madin-Darby canine kidney cells. Müsch, A., Cohen, D., Yeaman, C., Nelson, W.J., Rodriguez-Boulan, E., Brennwald, P.J. Mol. Biol. Cell (2002) [Pubmed]
  3. The human homologue of the murine Llglh gene (LLGL) maps within the Smith-Magenis syndrome region in 17p11.2. Koyama, K., Fukushima, Y., Inazawa, J., Tomotsune, D., Takahashi, N., Nakamura, Y. Cytogenet. Cell Genet. (1996) [Pubmed]
  4. Fliih, the murine homologue of the Drosophila melanogaster flightless I gene: nucleotide sequence, chromosomal mapping and overlap with Llglh. Campbell, H.D., Fountain, S., Young, I.G., Weitz, S., Lichter, P., Hoheisel, J.D. DNA Seq. (2000) [Pubmed]
  5. The WD-40 repeat motif of Lgl tumor suppressor proteins associated with salt tolerance and temperature sensitivity. Kim, Y.K., Kim, Y.S., Baek, K.H. Biochem. Biophys. Res. Commun. (2005) [Pubmed]
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