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Gene Review:

Atn1 - atrophin 1

Rattus norvegicus

Synonyms: Atrophin-1, Dentatorubral-pallidoluysian atrophy protein homolog, Drpla

Loev, S.J. et al., Kariya, S. et al., Kariya, S. et al., Schmitt, I. et al.

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Disease relevance of Atn1

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal-dominant neurodegenerative disorder caused by expansion of CAG repeats in the DRPLA gene, which codes for a polyglutamine (polyQ) stretch [1].

Psychiatry related information on Atn1

Dentatorubral pallidoluysian atrophy (DRPLA) is a rare, progressive, fatal neuropsychiatric disorder similar to Huntington's disease, caused by an expansion of a CAG trinucleotide repeat encoding glutamine [2].

High impact information on Atn1

Hardly anything is known about the physiological function of the DRPLA gene and the pathological mechanisms causing neuronal cell death and leading to these symptoms [3].
The widespread expression of this gene is consistent with the possibility that DRPLA, like other glutamine repeat diseases, is a result of an abnormality at the protein level [2].
Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurological disorder caused by a CAG repeat expansion in the DRPLA gene encoding polyglutamine (polyQ) [4].
Humanin attenuates apoptosis induced by DRPLA proteins with expanded polyglutamine stretches [1].

References

Humanin attenuates apoptosis induced by DRPLA proteins with expanded polyglutamine stretches. Kariya, S., Hirano, M., Nagai, Y., Furiya, Y., Fujikake, N., Toda, T., Ueno, S. J. Mol. Neurosci. (2005) [Pubmed]
Cloning and expression of the rat atrophin-I (DRPLA disease gene) homologue. Loev, S.J., Margolis, R.L., Young, W.S., Li, S.H., Schilling, G., Ashworth, R.G., Ross, C.A. Neurobiol. Dis. (1995) [Pubmed]
Predominant neuronal expression of the gene responsible for dentatorubral-pallidoluysian atrophy (DRPLA) in rat. Schmitt, I., Epplen, J.T., Riess, O. Hum. Mol. Genet. (1995) [Pubmed]
Cytoprotective effect of novel histone deacetylase inhibitors against polyglutamine toxicity. Kariya, S., Hirano, M., Uesato, S., Nagai, Y., Nagaoka, Y., Furiya, Y., Asai, H., Fujikake, N., Toda, T., Ueno, S. Neurosci. Lett. (2006) [Pubmed]

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ATN1	Bos taurus
ATN1	Canis lupus familiaris
ATN1	Gallus gallus
ATN1	Homo sapiens
ATN1	Macaca mulatta
Atn1	Mus musculus
ATN1	Pan troglodytes
LOC100911672	Rattus norvegicus

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