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Pupillary light reflex deficits in a canine model of late infantile neuronal ceroid lipofuscinosis.

Whiting RE, Narfström K, Yao G, Pearce JW, Coates JR, Castaner LJ, Katz ML.

Exp Eye Res. 2013 Nov;116:402-10. doi: 10.1016/j.exer.2013.10.006. Epub 2013 Oct 14.


Enzyme replacement therapy delays pupillary light reflex deficits in a canine model of late infantile neuronal ceroid lipofuscinosis.

Whiting RE, Narfström K, Yao G, Pearce JW, Coates JR, Castaner LJ, Jensen CA, Dougherty BN, Vuillemenot BR, Kennedy D, O'Neill CA, Katz ML.

Exp Eye Res. 2014 Aug;125:164-72. doi: 10.1016/j.exer.2014.06.008. Epub 2014 Jun 19.


Multifocal retinopathy in Dachshunds with CLN2 neuronal ceroid lipofuscinosis.

Whiting RE, Pearce JW, Castaner LJ, Jensen CA, Katz RJ, Gilliam DH, Katz ML.

Exp Eye Res. 2015 May;134:123-32. doi: 10.1016/j.exer.2015.02.012. Epub 2015 Feb 16.


Retinal pathology in a canine model of late infantile neuronal ceroid lipofuscinosis.

Katz ML, Coates JR, Cooper JJ, O'Brien DP, Jeong M, Narfström K.

Invest Ophthalmol Vis Sci. 2008 Jun;49(6):2686-95. doi: 10.1167/iovs.08-1712. Epub 2008 Mar 14.


Intracerebroventricular gene therapy that delays neurological disease progression is associated with selective preservation of retinal ganglion cells in a canine model of CLN2 disease.

Whiting RE, Jensen CA, Pearce JW, Gillespie LE, Bristow DE, Katz ML.

Exp Eye Res. 2016 May;146:276-82. doi: 10.1016/j.exer.2016.03.023. Epub 2016 Apr 1.


Quantitative assessment of the canine pupillary light reflex.

Whiting RE, Yao G, Narfström K, Pearce JW, Coates JR, Dodam JR, Castaner LJ, Katz ML.

Invest Ophthalmol Vis Sci. 2013 Aug 13;54(8):5432-40. doi: 10.1167/iovs.13-12012.


Ocular phenotype in a mouse gene knockout model for infantile neuronal ceroid lipofuscinosis.

Lei B, Tullis GE, Kirk MD, Zhang K, Katz ML.

J Neurosci Res. 2006 Oct;84(5):1139-49.


Evaluation of retinal status using chromatic pupil light reflex activity in healthy and diseased canine eyes.

Grozdanic SD, Matic M, Sakaguchi DS, Kardon RH.

Invest Ophthalmol Vis Sci. 2007 Nov;48(11):5178-83.


A reversal learning task detects cognitive deficits in a Dachshund model of late-infantile neuronal ceroid lipofuscinosis.

Sanders DN, Kanazono S, Wininger FA, Whiting RE, Flournoy CA, Coates JR, Castaner LJ, O'Brien DP, Katz ML.

Genes Brain Behav. 2011 Oct;10(7):798-804. doi: 10.1111/j.1601-183X.2011.00718.x. Epub 2011 Aug 7.


Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis.

Katz ML, Johnson GS, Tullis GE, Lei B.

Neurobiol Dis. 2008 Feb;29(2):242-53. Epub 2007 Sep 7.


Intravitreal implantation of TPP1-transduced stem cells delays retinal degeneration in canine CLN2 neuronal ceroid lipofuscinosis.

Tracy CJ, Whiting RE, Pearce JW, Williamson BG, Vansteenkiste DP, Gillespie LE, Castaner LJ, Bryan JN, Coates JR, Jensen CA, Katz ML.

Exp Eye Res. 2016 Nov;152:77-87. doi: 10.1016/j.exer.2016.09.003. Epub 2016 Sep 13.


Enzyme replacement therapy attenuates disease progression in a canine model of late-infantile neuronal ceroid lipofuscinosis (CLN2 disease).

Katz ML, Coates JR, Sibigtroth CM, Taylor JD, Carpentier M, Young WM, Wininger FA, Kennedy D, Vuillemenot BR, O'Neill CA.

J Neurosci Res. 2014 Nov;92(11):1591-8. doi: 10.1002/jnr.23423. Epub 2014 Jun 17.


Two-color pupillometry in enhanced S-cone syndrome caused by NR2E3 mutations.

Collison FT, Park JC, Fishman GA, Stone EM, McAnany JJ.

Doc Ophthalmol. 2016 Jun;132(3):157-66. doi: 10.1007/s10633-016-9535-0. Epub 2016 Mar 31.


Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze.

Wendt KD, Lei B, Schachtman TR, Tullis GE, Ibe ME, Katz ML.

Behav Brain Res. 2005 Jun 20;161(2):175-82.


Electroretinographic and clinicopathologic correlations of retinal dysfunction in infantile neuronal ceroid lipofuscinosis (infantile Batten disease).

Weleber RG, Gupta N, Trzupek KM, Wepner MS, Kurz DE, Milam AH.

Mol Genet Metab. 2004 Sep-Oct;83(1-2):128-37.


Ultraviolet light-induced and green light-induced transient pupillary light reflex in mice.

Yao G, Zhang K, Bellassai M, Chang B, Lei B.

Curr Eye Res. 2006 Nov;31(11):925-33.


Neuronal ceroid lipofuscinosis associated with an MFSD8 mutation in Chihuahuas.

Ashwini A, D'Angelo A, Yamato O, Giordano C, Cagnotti G, Harcourt-Brown T, Mhlanga-Mutangadura T, Guo J, Johnson GS, Katz ML.

Mol Genet Metab. 2016 Aug;118(4):326-32. doi: 10.1016/j.ymgme.2016.05.008. Epub 2016 May 13.


A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration.

Sleat DE, Wiseman JA, El-Banna M, Kim KH, Mao Q, Price S, Macauley SL, Sidman RL, Shen MM, Zhao Q, Passini MA, Davidson BL, Stewart GR, Lobel P.

J Neurosci. 2004 Oct 13;24(41):9117-26.

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