OMIA:000177-9615 : Cerebellar cortical atrophy in Canis lupus familiaris (dog)

In other species: domestic cat , taurine cattle , sheep

Categories: Nervous system phene

Mendelian trait/disorder: yes

Mode of inheritance: Autosomal recessive

Considered a defect: yes

Key variant known: no

Cross-species summary: Wasting (decrease) of the outer layer of the grey matter of the cerebellum (the part of the brain situated behind the two hemispheres and above the pons and medulla oblongata).

Inheritance: Olby et a. (2004) provided reasonable evidence supporting autosomal recessive inheritance in a family of American Staffordshire Terriers. A likely disease causing mutation has been identified in this breed - see 'lysosomal storage disease, ARSG related' (OMIA001503-9615) for further details.

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2023). OMIA:000177-9615: 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 Stee, K., Van Poucke, M., Lowrie, M., Van Ham, L., Peelman, L., Olby, N., Bhatti, S.F.M. :
Phenotypic and genetic aspects of hereditary ataxia in dogs. J Vet Intern Med 37:1306-1322, 2023. Pubmed reference: 37341581. DOI: 10.1111/jvim.16742.
2007 Nibe, K., Kita, C., Morozumi, M., Awamura, Y., Tamura, S., Okuno, S., Kobayashi, T., Uchida, K. :
Clinicopathological features of canine neuroaxonal dystrophy and cerebellar cortical abiotrophy in Papillon and Papillon-related dogs. J Vet Med Sci 69:1047-52, 2007. Pubmed reference: 17984592.
2006 Buijtels, J.J., Kroeze, E.J., Voorhout, G., Schellens, C.J., van Nes, J.J. :
[Cerebellar cortical degeneration in an American Staffordshire terrier]. Tijdschr Diergeneeskd 131:518-22, 2006. Pubmed reference: 16916197.
2004 Olby, N., Blot, S., Thibaud, JL., Phillips, J., O'Brien, DP., Burr, J., Berg, J., Brown, T., Breen, M. :
Cerebellar cortical degeneration in adult American Staffordshire Terriers. J Vet Intern Med 18:201-8, 2004. Pubmed reference: 15058771.
2003 Hanzlicek, D., Kathmann, I., Bley, T., Srenk, P., Botteron, C., Gaillard, C., Jaggy, A. :
[Cerebellar cortical abiotrophy in American Staffordshire terriers: clinical and pathological description of 3 cases] Schweizer Archiv fur Tierheilkunde 145:369-75, 2003. Pubmed reference: 12951908.
2000 van, Tongeren, S.E., van, Vonderen, I.K., van, Nes, J.J., van, den, Ingh, T.S.G.A.M. :
Cerebellar cortical abiotrophy in two Portuguese Podenco littermates Veterinary Quarterly 22:172-174, 2000. Pubmed reference: 10952450.
1991 Perille, A.L., Baer, K., Joseph, R.J., Carrillo, J.M., Averill, D.R. :
Postnatal cerebellar cortical degeneration in Labrador Retriever puppies. Canadian Veterinary Journal - Revue Veterinaire Canadienne 32:619-621, 1991.

Edit History

  • Created by Frank Nicholas on 27 Oct 2005
  • Changed by Imke Tammen2 on 08 Jan 2021
  • Changed by Imke Tammen2 on 23 Jun 2023