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Items: 1 to 20 of 95

1.

Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease.

Gulinello M, Chen F, Dobrenis K.

Behav Brain Res. 2008 Nov 21;193(2):315-9. doi: 10.1016/j.bbr.2008.06.016. Epub 2008 Jun 20.

2.

Bi-phasic gliosis drives neuropathology in a Sandhoff disease mouse model.

Hooper AW, Igdoura SA.

J Neuroimmunol. 2016 Oct 15;299:19-27. doi: 10.1016/j.jneuroim.2016.08.008. Epub 2016 Aug 8.

PMID:
27725117
3.

Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice.

Denny CA, Kasperzyk JL, Gorham KN, Bronson RT, Seyfried TN.

J Neurosci Res. 2006 May 1;83(6):1028-38.

PMID:
16521125
4.

Long-term correction of Sandhoff disease following intravenous delivery of rAAV9 to mouse neonates.

Walia JS, Altaleb N, Bello A, Kruck C, LaFave MC, Varshney GK, Burgess SM, Chowdhury B, Hurlbut D, Hemming R, Kobinger GP, Triggs-Raine B.

Mol Ther. 2015 Mar;23(3):414-22. doi: 10.1038/mt.2014.240. Epub 2014 Dec 17.

5.

Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.

Jeyakumar M, Butters TD, Cortina-Borja M, Hunnam V, Proia RL, Perry VH, Dwek RA, Platt FM.

Proc Natl Acad Sci U S A. 1999 May 25;96(11):6388-93.

6.

Peripheral neuropathy in a family with Sandhoff disease and SH3TC2 deficiency.

Grunseich C, Schindler AB, Chen KL, Bakar D, Mankodi A, Traslavina R, Ray-Chaudhury A, Lehky TJ, Baker EH, Maragakis NJ, Tifft CJ, Fischbeck KH.

J Neurol. 2015;262(4):1066-8. doi: 10.1007/s00415-015-7683-x. Epub 2015 Mar 4. No abstract available.

7.

[Molecular pathogenesis and therapeutic approach of GM2 gangliosidosis].

Tsuji D.

Yakugaku Zasshi. 2013;133(2):269-74. Review. Japanese.

8.

Neuronal pentraxin 1 depletion delays neurodegeneration and extends life in Sandhoff disease mice.

Hooper AWM, Alamilla JF, Venier RE, Gillespie DC, Igdoura SA.

Hum Mol Genet. 2017 Feb 15;26(4):661-673. doi: 10.1093/hmg/ddw422.

PMID:
28007910
9.

The trigeminal retrograde transfer pathway in the treatment of neurodegeneration.

Kyrkanides S, Yang M, Tallents RH, Miller JN, Brouxhon SM, Olschowka JA.

J Neuroimmunol. 2009 Apr 30;209(1-2):139-42. doi: 10.1016/j.jneuroim.2009.02.006. Epub 2009 Mar 10.

PMID:
19278737
10.

Impaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff disease.

Ogawa Y, Tanaka M, Tanabe M, Suzuki T, Togawa T, Fukushige T, Kanekura T, Sakuraba H, Oishi K.

PLoS One. 2013;8(1):e55856. doi: 10.1371/journal.pone.0055856. Epub 2013 Jan 31.

11.

Characterization of the mutant β-subunit of β-hexosaminidase for dimer formation responsible for the adult form of Sandhoff disease with the motor neuron disease phenotype.

Yamada K, Takado Y, Kato YS, Yamada Y, Ishiguro H, Wakamatsu N.

J Biochem. 2013 Jan;153(1):111-9. doi: 10.1093/jb/mvs131. Epub 2012 Nov 5.

12.

Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease.

Van Raamsdonk JM, Pearson J, Slow EJ, Hossain SM, Leavitt BR, Hayden MR.

J Neurosci. 2005 Apr 20;25(16):4169-80.

13.

Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.

McNally MA, Baek RC, Avila RL, Seyfried TN, Strichartz GR, Kirschner DA.

J Negat Results Biomed. 2007 Jul 10;6:8.

14.

A semicircular controlled cortical impact produces long-term motor and cognitive dysfunction that correlates well with damage to both the sensorimotor cortex and hippocampus.

Liu NK, Zhang YP, Zou J, Verhovshek T, Chen C, Lu QB, Walker CL, Shields CB, Xu XM.

Brain Res. 2014 Aug 12;1576:18-26. doi: 10.1016/j.brainres.2014.05.042. Epub 2014 Jun 4.

PMID:
24905625
15.

Progressive impairment in olfactory working memory in a mouse model of Mild Cognitive Impairment.

Young JW, Sharkey J, Finlayson K.

Neurobiol Aging. 2009 Sep;30(9):1430-43. doi: 10.1016/j.neurobiolaging.2007.11.018. Epub 2008 Feb 1.

PMID:
18242780
16.

New blood cells help an overburdened brain.

Mattson MP.

Trends Neurosci. 2001 Jan;24(1):5. No abstract available.

PMID:
11163874
17.

New cases of adult-onset Sandhoff disease with a cerebellar or lower motor neuron phenotype.

Delnooz CC, Lefeber DJ, Langemeijer SM, Hoffjan S, Dekomien G, Zwarts MJ, Van Engelen BG, Wevers RA, Schelhaas HJ, van de Warrenburg BP.

J Neurol Neurosurg Psychiatry. 2010 Sep;81(9):968-72. doi: 10.1136/jnnp.2009.177089.

PMID:
20798201
18.

Miglustat therapy in juvenile Sandhoff disease.

Tallaksen CM, Berg JE.

J Inherit Metab Dis. 2009 Dec;32 Suppl 1:S289-93. doi: 10.1007/s10545-009-1224-7. Epub 2009 Nov 4.

PMID:
19898953
19.

Incidence and carrier frequency of Sandhoff disease in Saskatchewan determined using a novel substrate with detection by tandem mass spectrometry and molecular genetic analysis.

Fitterer B, Hall P, Antonishyn N, Desikan R, Gelb M, Lehotay D.

Mol Genet Metab. 2014 Mar;111(3):382-9. doi: 10.1016/j.ymgme.2014.01.002. Epub 2014 Jan 13.

20.

Motor, emotional and cognitive deficits in adult BACHD mice: a model for Huntington's disease.

Abada YS, Schreiber R, Ellenbroek B.

Behav Brain Res. 2013 Feb 1;238:243-51. doi: 10.1016/j.bbr.2012.10.039. Epub 2012 Oct 30.

PMID:
23123142

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