@article{urkasemsin_nielsen_singleton_arepalli_hernandez_agler_olby_2017, title={Genetics of Hereditary Ataxia in Scottish Terriers}, volume={31}, ISSN={0891-6640}, url={http://dx.doi.org/10.1111/jvim.14738}, DOI={10.1111/jvim.14738}, abstractNote={BackgroundScottish Terriers have a high incidence of juvenile onset hereditary ataxia primarily affecting the Purkinje neuron of the cerebellar cortex and causing slowly progressive cerebellar dysfunction.}, number={4}, journal={Journal of Veterinary Internal Medicine}, publisher={Wiley}, author={Urkasemsin, G. and Nielsen, D.M. and Singleton, A. and Arepalli, S. and Hernandez, D. and Agler, C. and Olby, N.J.}, year={2017}, month={May}, pages={1132–1139} }
 @article{urkasemsin_olby_2015, title={Clinical characteristics of Scottie Cramp in 31 cases}, volume={56}, ISSN={0022-4510}, url={http://dx.doi.org/10.1111/jsap.12317}, DOI={10.1111/jsap.12317}, abstractNote={OBJECTIVETo report the clinical features, with response to therapy and long‐term outcome of Scottie Cramp as described by owners.}, number={4}, journal={Journal of Small Animal Practice}, publisher={Wiley}, author={Urkasemsin, G. and Olby, N. J.}, year={2015}, month={Jan}, pages={276–280} }
 @article{agler_nielsen_urkasemsin_singleton_tonomura_sigurdsson_tang_linder_arepalli_hernandez_et al._2014, title={Canine hereditary ataxia in Old English Sheepdogs and Gordon Setters is associated with a defect in the autophagy gene encoding RAB24}, volume={10}, number={2}, journal={PLoS Genetics}, author={Agler, C. and Nielsen, D. M. and Urkasemsin, G. and Singleton, A. and Tonomura, N. and Sigurdsson, S. and Tang, R. Q. and Linder, K. and Arepalli, S. and Hernandez, D. and et al.}, year={2014} }
 @article{ferreira_padilla_urkasemsin_yoon_goeckner_hu_ko_2013, title={Titanium-enriched hydroxyapatite-gelatin scaffolds with osteogenically differentiated progenitor cell aggregates for calvaria bone regeneration}, volume={19}, number={15-16}, journal={Tissue Engineering. Part A}, author={Ferreira, J. R. and Padilla, R. and Urkasemsin, G. and Yoon, K. and Goeckner, K. and Hu, W. S. and Ko, C. C.}, year={2013}, pages={1803–1816} }
 @article{urkasemsin_linder_bell_lahunta_olby_2012, title={Mapping of Purkinje Neuron Loss and Polyglucosan Body Accumulation in Hereditary Cerebellar Degeneration in Scottish Terriers}, volume={49}, ISSN={["1544-2217"]}, DOI={10.1177/0300985811412622}, abstractNote={ A hereditary cerebellar degenerative disorder has emerged in Scottish Terriers. The aims of this study were to describe and quantify polyglucosan body accumulation and quantify Purkinje neurons in the cerebellum of affected and control dogs. The brains of 6 affected Scottish Terriers ranging in age from 8 to 15 years and 8 age-matched control dogs were examined histopathologically. Counts of Purkinje neurons and polyglucosan bodies were performed in control and affected dogs on cerebellar sections stained with periodic acid–Schiff. Affected dogs showed a significant loss of Purkinje neurons compared with control dogs (vermis: P < .0001; hemisphere: P = .0104). The degeneration was significantly more pronounced dorsally than ventrally ( P < .0001). There were significantly more polyglucosan bodies in the ventral half of the vermis when compared with the dorsal half ( P < .0001) in affected dogs. In addition, there were more polyglucosan bodies in the ventral half of the vermis in affected dogs than in control dogs ( P = .0005). Polyglucosan bodies in all affected dogs stained positively with toluidine blue and alcian blue. Immunohistochemically, polyglucosan bodies in affected dogs were positive for neurofilament 200 kD and ubiquitin and negative for glial fibrillary acidic protein, synaptophysin, neurospecific enolase, vimentin, and S100; the bodies were negative for all antigens in control dogs. Ultrastructurally, polyglucosan bodies in 1 affected dog were non–membrane-bound, amorphous structures with a dense core. This study demonstrates significant Purkinje cell loss and increased polyglucosan bodies in the cerebellum of affected Scottish Terriers. }, number={5}, journal={VETERINARY PATHOLOGY}, author={Urkasemsin, G. and Linder, K. E. and Bell, J. S. and Lahunta, A. and Olby, N. J.}, year={2012}, month={Sep}, pages={852–859} }
 @article{urkasemsin_linder_bell_lahunta_olby_2010, title={Hereditary Cerebellar Degeneration in Scottish Terriers}, volume={24}, ISSN={["1939-1676"]}, DOI={10.1111/j.1939-1676.2010.0499.x}, abstractNote={BACKGROUND
Hereditary cerebellar degeneration is described in several dog breeds. This heterogeneous group of diseases causes cerebellar ataxia associated with cerebellar cortical degeneration.


OBJECTIVE
To report the clinical and histopathological features, and describe the mode of inheritance of hereditary cerebellar degeneration in Scottish Terriers.


ANIMALS
Sixty-two affected dogs recruited through the Scottish Terrier Club of America.


MATERIALS AND METHODS
Prospective, observational study: Owners of affected dogs were contacted for a description of clinical signs, age of onset, and disease progression. Medical records, videotapes of gait, and brain imaging were evaluated. When possible, necropsy was performed and the brain examined histopathologically. Prevalence of the disease was estimated and a pedigree analysis was performed to determine mode of inheritance.


RESULTS
Gait abnormalities were noted in the 1st year of life in 76% of dogs, and progressed slowly; only 1 of 27 dogs dead at time of writing was euthanized because of cerebellar degeneration. Clinical signs included wide based stance, dysmetria, intention tremor, and difficulty negotiating stairs and running. Cerebellar atrophy was detected on magnetic resonance imaging. On histopathological examination, there was segmental loss of Purkinje neurons, thinning of molecular and granular layers, and polyglucosan bodies in the molecular layer. Prevalence of disease was estimated at 1 in 1,335 American Kennel Club registered Scottish Terriers. Genetic analysis results are consistent with an autosomal recessive mode of inheritance.


CONCLUSION AND CLINICAL IMPORTANCE
A hereditary cerebellar degenerative disorder with a relatively mild phenotype has emerged in the Scottish Terrier. Genetic studies are needed.}, number={3}, journal={JOURNAL OF VETERINARY INTERNAL MEDICINE}, author={Urkasemsin, G. and Linder, K. E. and Bell, J. S. and Lahunta, A. and Olby, N. J.}, year={2010}, pages={565–570} }