OMIA:002069-10036 : Dystonia, generic in Mesocricetus auratus (golden hamster)

In other species: dog , domestic cat

Categories: Nervous system phene

Mendelian trait/disorder: yes

Mode of inheritance: Autosomal recessive

Considered a defect: yes

Key variant known: no

Species-specific name: idiopathic paroxysmal dystonia

Species-specific symbol: dt^SZ

History: The occurrence of this mutant hamster phene was first described by Yoon et al. (1976).

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2022). OMIA:002069-10036: Online Mendelian Inheritance in Animals (OMIA) [dataset].


Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.

2023 Lüttig, A., Perl, S., Zetsche, M., Richter, F., Franz, D., Heerdegen, M., Köhling, R., Richter, A. :
Short-term stimulations of the entopeduncular nucleus induce cerebellar changes of c-Fos expression in an animal model of paroxysmal dystonia. Brain Res :148672, 2023. Pubmed reference: 37956748. DOI: 10.1016/j.brainres.2023.148672.
2021 Knorr, S., Musacchio, T., Paulat, R., Matthies, C., Endres, H., Wenger, N., Harms, C., Ip, C.W. :
Experimental deep brain stimulation in rodent models of movement disorders. Exp Neurol :113926, 2021. Pubmed reference: 34793784. DOI: 10.1016/j.expneurol.2021.113926.
Paap, M., Perl, S., Lüttig, A., Plocksties, F., Niemann, C., Timmermann, D., Bahls, C., van Rienen, U., Franz, D., Zwar, M., Rohde, M., Köhling, R., Richter, A. :
Deep brain stimulation by optimized stimulators in a phenotypic model of dystonia: Effects of different frequencies. Neurobiol Dis 147:105163, 2021. Pubmed reference: 33166698. DOI: 10.1016/j.nbd.2020.105163.
2017 Bode, C., Richter, F., Spröte, C., Brigadski, T., Bauer, A., Fietz, S., Fritschy, J.M., Richter, A. :
Altered postnatal maturation of striatal GABAergic interneurons in a phenotypic animal model of dystonia. Exp Neurol 287:44-53, 2017. Pubmed reference: 27780732. DOI: 10.1016/j.expneurol.2016.10.013.
2016 Spröte, C., Richter, F., Bauer, A., Gerstenberger, J., Richter, A. :
The α2β3γ2 GABAA receptor preferring agonist NS11394 aggravates dystonia in the phenotypic dt(sz) model. Eur J Pharmacol 791:655-658, 2016. Pubmed reference: 27693801. DOI: 10.1016/j.ejphar.2016.09.040.
2010 Leblois, A., Reese, R., Labarre, D., Hamann, M., Richter, A., Boraud, T., Meissner, W.G. :
Deep brain stimulation changes basal ganglia output nuclei firing pattern in the dystonic hamster. Neurobiol Dis 38:288-98, 2010. Pubmed reference: 20138992. DOI: 10.1016/j.nbd.2010.01.020.
2009 Reese, R., Charron, G., Nadjar, A., Aubert, I., Thiolat, M.L., Hamann, M., Richter, A., Bezard, E., Meissner, W.G. :
High frequency stimulation of the entopeduncular nucleus sets the cortico-basal ganglia network to a new functional state in the dystonic hamster. Neurobiol Dis 35:399-405, 2009. Pubmed reference: 19501651. DOI: 10.1016/j.nbd.2009.05.022.
Sander, S.E., Richter, F., Raymond, R., Diwan, M., Lange, N., Nobrega, J.N., Richter, A. :
Pharmacological and autoradiographic studies on the pathophysiological role of GABA(B) receptors in the dystonic hamster: pronounced antidystonic effects of baclofen after striatal injections. Neuroscience 162:423-30, 2009. Pubmed reference: 19427369. DOI: 10.1016/j.neuroscience.2009.05.007.
2008 Hamann, M., Sander, S.E., Richter, A. :
Brivaracetam and seletracetam, two new SV2A ligands, improve paroxysmal dystonia in the dt sz mutant hamster. Eur J Pharmacol 601:99-102, 2008. Pubmed reference: 19014930. DOI: 10.1016/j.ejphar.2008.10.048.
2007 Hamann, M., Richter, A., Meillasson, F.V., Nitsch, C., Ebert, U. :
Age-related changes in parvalbumin-positive interneurons in the striatum, but not in the sensorimotor cortex in dystonic brains of the dt(sz) mutant hamster. Brain Res 1150:190-9, 2007. Pubmed reference: 17391652. DOI: 10.1016/j.brainres.2007.02.074.
2004 Harnack, D., Hamann, M., Meissner, W., Morgenstern, R., Kupsch, A., Richter, A. :
High-frequency stimulation of the entopeduncular nucleus improves dystonia in dtsz hamsters. Neuroreport 15:1391-3, 2004. Pubmed reference: 15194859. DOI: 10.1097/01.wnr.0000130435.75715.64.
2002 Richter, A., Löscher, W. :
Animal models of paroxysmal dystonia. Adv Neurol 89:443-51, 2002. Pubmed reference: 11968468.
2000 Richter, A., Löscher, W. :
Animal models of dystonia. Funct Neurol 15:259-67, 2000. Pubmed reference: 11213529.
1998 Richter, A., Löscher, W. :
Pathology of idiopathic dystonia: findings from genetic animal models. Prog Neurobiol 54:633-77, 1998. Pubmed reference: 9560845.
1991 Fredow, G., Löscher, W. :
Effects of pharmacological manipulation of GABAergic neurotransmission in a new mutant hamster model of paroxysmal dystonia. Eur J Pharmacol 192:207-19, 1991. Pubmed reference: 1851702. DOI: 10.1016/0014-2999(91)90045-r.
1990 Wahnschaffe, U., Fredow, G., Heintz, P., Löscher, W. :
Neuropathological studies in a mutant hamster model of paroxysmal dystonia. Mov Disord 5:286-93, 1990. Pubmed reference: 2259352. DOI: 10.1002/mds.870050405.
1989 Löscher, W., Fisher, J.E., Schmidt, D., Fredow, G., Hönack, D., Iturrian, W.B. :
The sz mutant hamster: a genetic model of epilepsy or of paroxysmal dystonia? Mov Disord 4:219-32, 1989. Pubmed reference: 2779593. DOI: 10.1002/mds.870040304.
1979 Yoon, C.H., Peterson, J.S. :
Linkage group IV in the Syrian hamster: spontaneous seizure and jute. J Hered 70:279-80, 1979. Pubmed reference: 489957. DOI: 10.1093/oxfordjournals.jhered.a109257.
1976 Yoon, C.H., Peterson, J.S., Corrow, D. :
Spontaneous seizures: a new mutation in Syrian golden hamsters. J Hered 67:115-6, 1976. Pubmed reference: 1270790. DOI: 10.1093/oxfordjournals.jhered.a108676.

Edit History

  • Created by Frank Nicholas on 31 Oct 2016
  • Changed by Imke Tammen2 on 24 Nov 2021
  • Changed by Frank Nicholas on 12 May 2022