claw paw


Gene Symbol: claw paw
Description: leucine-rich repeat LGI family, member 4
Alias: Lgil3, clp, leucine-rich repeat LGI family member 4, LGI1-like protein 3, leucine-rich glioma inactivated protein 4
Species: mouse
Products:     claw paw

Top Publications

  1. Bermingham J, Shumas S, Whisenhunt T, Sirkowski E, O Connell S, Scherer S, et al. Identification of genes that are downregulated in the absence of the POU domain transcription factor pou3f1 (Oct-6, Tst-1, SCIP) in sciatic nerve. J Neurosci. 2002;22:10217-31 pubmed
    ..pou3f1 appears to require the claw paw gene product for activation of at least some of its downstream effector genes...
  2. Staub E, Perez Tur J, Siebert R, Nobile C, Moschonas N, Deloukas P, et al. The novel EPTP repeat defines a superfamily of proteins implicated in epileptic disorders. Trends Biochem Sci. 2002;27:441-4 pubmed
    ..Genes encoding proteins of this family are located in genomic regions associated with epilepsy and other neurological disorders. ..
  3. Henry E, Eicher E, Sidman R. The mouse mutation claw paw: forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered. 1991;82:287-94 pubmed
    We describe a murine autosomal recessive mutation claw paw (gene symbol clp), which in homozygous clp/clp mice produces striking abnormalities of limb posture within the first one or two postnatal days...
  4. Ozkaynak E, Abello G, Jaegle M, van Berge L, Hamer D, Kegel L, et al. Adam22 is a major neuronal receptor for Lgi4-mediated Schwann cell signaling. J Neurosci. 2010;30:3857-64 pubmed publisher an important regulator of myelination in the PNS, and its dysfunction results in arthrogryposis as observed in claw paw mice...
  5. Nishino J, Saunders T, Sagane K, Morrison S. Lgi4 promotes the proliferation and differentiation of glial lineage cells throughout the developing peripheral nervous system. J Neurosci. 2010;30:15228-40 pubmed publisher regulate peripheral nerve myelination (having been identified as the mutated gene in spontaneously arising claw paw mutant mice), but Lgi4 is not known to play any role in PNS development outside of peripheral nerves...
  6. Kegel L, Jaegle M, Driegen S, Aunin E, Leslie K, Fukata Y, et al. Functional phylogenetic analysis of LGI proteins identifies an interaction motif crucial for myelination. Development. 2014;141:1749-56 pubmed publisher
  7. Niemann S, Sidman R, Nave K. Evidence against altered forms of MAG in the dysmyelinated mouse mutant claw paw. Mamm Genome. 1998;9:903-4 pubmed
  8. Darbas A, Jaegle M, Walbeehm E, van den Burg H, Driegen S, Broos L, et al. Cell autonomy of the mouse claw paw mutation. Dev Biol. 2004;272:470-82 pubmed
    Mice homozygous for the autosomal recessive mutation claw paw (clp) are characterized by limb posture abnormalities and congenital hypomyelination, with delayed onset of myelination of the peripheral nervous system but not the central ..
  9. Bermingham J, Shearin H, Pennington J, O Moore J, Jaegle M, Driegen S, et al. The claw paw mutation reveals a role for Lgi4 in peripheral nerve development. Nat Neurosci. 2006;9:76-84 pubmed
    ..The delayed axonal sorting and hypomyelination throughout the peripheral nervous system of claw paw (clp) mutant mice suggest that the clp gene product is critical for these interactions...

More Information


  1. Herranz PĂ©rez V, Olucha Bordonau F, Morante Redolat J, Perez Tur J. Regional distribution of the leucine-rich glioma inactivated (LGI) gene family transcripts in the adult mouse brain. Brain Res. 2010;1307:177-94 pubmed publisher
    ..Finally, LGI3 gene widespread expression in the brain suggests that its transcripts might be involved in a common cellular process present in different neuronal types. ..
  2. Koszowski A, Owens G, Levinson S. The effect of the mouse mutation claw paw on myelination and nodal frequency in sciatic nerves. J Neurosci. 1998;18:5859-68 pubmed
    ..We chose to study myelination and node of Ranvier formation in the hypomyelinating mouse mutant claw paw (clp). The phenotype of clp is delayed myelination in the peripheral nervous system...
  3. Scheel H, Tomiuk S, Hofmann K. A common protein interaction domain links two recently identified epilepsy genes. Hum Mol Genet. 2002;11:1757-62 pubmed
    ..The EAR domain is likely to play an important role in the pathogenesis of epilepsy, either by binding to an unknown anti-epileptic ligand, or more likely by interfering with axon guidance or synaptogenesis. ..
  4. Runkel F, Michels M, Franz T. Fxyd3 and Lgi4 expression in the adult mouse: a case of endogenous antisense expression. Mamm Genome. 2003;14:665-72 pubmed
    ..These results demonstrate that Fxyd3 and Lgi4 transcripts potentially form double-stranded RNA molecules in many cell types in vivo, which may impact on their respective expression. ..