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Gene Review

Glra1  -  glycine receptor, alpha 1 subunit

Mus musculus

Synonyms: B230397M16Rik, Glycine receptor 48 kDa subunit, Glycine receptor strychnine-binding subunit, Glycine receptor subunit alpha-1, nmf11, ...
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Disease relevance of Glra1


High impact information on Glra1


Biological context of Glra1


Anatomical context of Glra1

  • As shown by [3H]strychnine binding and western blot analysis employing subunit-specific antibodies, spinal cord of homozygous oscillator mice was totally devoid of alpha1-polypeptide, characterizing the Glra1(spd-ot) gene as a functional null allele of Glra1 [15].
  • No differences were observed in the levels of the major CNS and PNS myelin proteins or lipids of spd/spd mice versus littermate controls, suggesting that, unlike several closely linked mutations, the spd mutation does not affect myelination [12].
  • Alternatively, glycinergic tonic inhibition of sensitizing structures (e.g. the amygdala) in the wildtype may be diminished in spd/spd mutants, thus leading to a high sensitization level [16].

Associations of Glra1 with chemical compounds


Other interactions of Glra1

  • Although no recombination was observed between spd and Anx-6 or Glr-1, no evidence was obtained for a lesion in either gene [10].
  • Patch-clamp recordings were performed from retinal slices of wild-type, GlyRalpha1-deficient (Glra1(spd-ot)) and GlyRalpha3-deficient (Glra3(-/-)) mice [20].

Analytical, diagnostic and therapeutic context of Glra1

  • Sixteen patients suffering from spasmodic torticollis were selected: in 2 patients, BoNT/A treatment continued to be effective, in 9 patients, the treatment effect was impaired, and in 5 patients, secondary treatment failure developed [21].
  • The C. gloriamaris spasmodic peptide has been synthesized, and the refolded polypeptide was shown to be biologically active using a mouse bioassay [9].
  • Crystalline botulinum toxin type A was licensed in December 1989 by the Food and Drug Administration for treatment of certain spasmodic muscle disorders following 10 or more years of experimental treatment on human volunteers [22].
  • Spastic (spa), spasmodic (spd), and oscillator (ot) mice have naturally occurring glycine receptor (GlyR) mutations, which manifest as motor deficits and an exaggerated "startle response." Using whole-cell recording in hypoglossal motoneurons, we compared the physiological mechanisms by which each mutation alters GlyR function [23].


  1. Treatment of cervical dystonia hand spasms and laryngeal dystonia with botulinum toxin. Lees, A.J., Turjanski, N., Rivest, J., Whurr, R., Lorch, M., Brookes, G. J. Neurol. (1992) [Pubmed]
  2. Resistance to botulinum toxin injections for spasmodic dysphonia. Smith, M.E., Ford, C.N. Arch. Otolaryngol. Head Neck Surg. (2000) [Pubmed]
  3. Naltrexone inhibits the persistent spasmodic dyskinesia induced by chronic intraperitoneal administration of iminodipropionitrile (IDPN). Cadet, J.L., Braun, T.L. Neuropeptides (1986) [Pubmed]
  4. Ortho-iodosobenzoic acid: its acute toxicity and neurobehavioral effects in mice. Liu, W.F., Wei, Y.C., Lee, J.D., Ma, C., Lin, C.H. Toxicol. Lett. (1989) [Pubmed]
  5. Pharmacological studies on coleonol, a hypotensive diterpene from Coleus forskohlii. Dubey, M.P., Srimal, R.C., Nityanand, S., Dhawan, B.N. Journal of ethnopharmacology. (1981) [Pubmed]
  6. A missense mutation in the gene encoding the alpha 1 subunit of the inhibitory glycine receptor in the spasmodic mouse. Ryan, S.G., Buckwalter, M.S., Lynch, J.W., Handford, C.A., Segura, L., Shiang, R., Wasmuth, J.J., Camper, S.A., Schofield, P., O'Connell, P. Nat. Genet. (1994) [Pubmed]
  7. Hyperekplexia Phenotype of Glycine Receptor alpha1 Subunit Mutant Mice Identifies Zn(2+) as an Essential Endogenous Modulator of Glycinergic Neurotransmission. Hirzel, K., M??ller, U., Latal, A.T., H??lsmann, S., Grudzinska, J., Seeliger, M.W., Betz, H., Laube, B. Neuron (2006) [Pubmed]
  8. A frameshift mutation in the mouse alpha 1 glycine receptor gene (Glra1) results in progressive neurological symptoms and juvenile death. Buckwalter, M.S., Cook, S.A., Davisson, M.T., White, W.F., Camper, S.A. Hum. Mol. Genet. (1994) [Pubmed]
  9. Structure of a novel P-superfamily spasmodic conotoxin reveals an inhibitory cystine knot motif. Miles, L.A., Dy, C.Y., Nielsen, J., Barnham, K.J., Hinds, M.G., Olivera, B.M., Bulaj, G., Norton, R.S. J. Biol. Chem. (2002) [Pubmed]
  10. Genetic mapping and evaluation of candidate genes for spasmodic, a neurological mouse mutation with abnormal startle response. Buckwalter, M.S., Testa, C.M., Noebels, J.L., Camper, S.A. Genomics (1993) [Pubmed]
  11. Increased startle responses in mice carrying mutations of glycine receptor subunit genes. Koch, M., Kling, C., Becker, C.M. Neuroreport (1996) [Pubmed]
  12. Spasmodic, a mutation on chromosome 11 in the mouse. Lane, P.W., Ganser, A.L., Kerner, A.L., White, W.F. J. Hered. (1987) [Pubmed]
  13. Nmf11 is a novel ENU-induced mutation in the mouse glycine receptor alpha 1 subunit. Traka, M., Seburn, K.L., Popko, B. Mamm. Genome (2006) [Pubmed]
  14. Altered inhibitory synaptic transmission in superficial dorsal horn neurones in spastic and oscillator mice. Graham, B.A., Schofield, P.R., Sah, P., Callister, R.J. J. Physiol. (Lond.) (2003) [Pubmed]
  15. The frameshift mutation oscillator (Glra1(spd-ot)) produces a complete loss of glycine receptor alpha1-polypeptide in mouse central nervous system. Kling, C., Koch, M., Saul, B., Becker, C.M. Neuroscience (1997) [Pubmed]
  16. Increased sensitization of acoustic startle response in spasmodic mice with a mutation of the glycine receptor alpha1-subunit gene. Plappert, C.F., Pilz, P.K., Becker, K., Becker, C.M., Schnitzler, H.U. Behav. Brain Res. (2001) [Pubmed]
  17. A single amino acid determines differences in ethanol actions on strychnine-sensitive glycine receptors. Mascia, M.P., Mihic, S.J., Valenzuela, C.F., Schofield, P.R., Harris, R.A. Mol. Pharmacol. (1996) [Pubmed]
  18. The spasmodic peptide defines a new conotoxin superfamily. Lirazan, M.B., Hooper, D., Corpuz, G.P., Ramilo, C.A., Bandyopadhyay, P., Cruz, L.J., Olivera, B.M. Biochemistry (2000) [Pubmed]
  19. Clonidine and prazosin block the iminodipropionitrile (IDPN)-induced spasmodic dyskinetic syndrome in mice. Cadet, J.L., Braun, T., Freed, W.J. Pharmacol. Biochem. Behav. (1987) [Pubmed]
  20. Characterization of the glycinergic input to bipolar cells of the mouse retina. Ivanova, E., Müller, U., Wässle, H. Eur. J. Neurosci. (2006) [Pubmed]
  21. Sudomotor testing predicts the presence of neutralizing botulinum A toxin antibodies. Birklein, F., Walther, D., Bigalke, H., Winterholler, M., Erbguth, F. Ann. Neurol. (2002) [Pubmed]
  22. Properties and use of botulinum toxin and other microbial neurotoxins in medicine. Schantz, E.J., Johnson, E.A. Microbiol. Rev. (1992) [Pubmed]
  23. Distinct physiological mechanisms underlie altered glycinergic synaptic transmission in the murine mutants spastic, spasmodic, and oscillator. Graham, B.A., Schofield, P.R., Sah, P., Margrie, T.W., Callister, R.J. J. Neurosci. (2006) [Pubmed]
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