PubMed

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.PMID: 17623103 [PubMed - indexed for MEDLINE]Free PMC ArticleFree textRelated citations

Inefficiency in GM2 ganglioside elimination by human lysosomal beta-hexosaminidase beta-subunit gene transfer to fibroblastic cell line derived from Sandhoff disease model mice.

Itakura T, Kuroki A, Ishibashi Y, Tsuji D, Kawashita E, Higashine Y, Sakuraba H, Yamanaka S, Itoh K.

Biol Pharm Bull. 2006 Aug;29(8):1564-9.PMID: 16880605 [PubMed - indexed for MEDLINE]Free ArticleRelated citations

Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff disease.

Baek RC, Martin DR, Cox NR, Seyfried TN.

Lipids. 2009 Mar;44(3):197-205. Epub 2008 Nov 26.PMID: 19034545 [PubMed - indexed for MEDLINE]Related citations

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 [PubMed - indexed for MEDLINE]Related citations

Specific induction of macrophage inflammatory protein 1-alpha in glial cells of Sandhoff disease model mice associated with accumulation of N-acetylhexosaminyl glycoconjugates.

Tsuji D, Kuroki A, Ishibashi Y, Itakura T, Kuwahara J, Yamanaka S, Itoh K.

J Neurochem. 2005 Mar;92(6):1497-507.PMID: 15748167 [PubMed - indexed for MEDLINE]Related citations

Metabolic correction in microglia derived from Sandhoff disease model mice.

Tsuji D, Kuroki A, Ishibashi Y, Itakura T, Itoh K.

J Neurochem. 2005 Sep;94(6):1631-8. Epub 2005 Aug 10.PMID: 16092933 [PubMed - indexed for MEDLINE]Related citations

N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice.

Baek RC, Kasperzyk JL, Platt FM, Seyfried TN.

Neurochem Int. 2008 May;52(6):1125-33. Epub 2007 Dec 8.PMID: 18207611 [PubMed - indexed for MEDLINE]Related citations

Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment.

Liu Y, Hoffmann A, Grinberg A, Westphal H, McDonald MP, Miller KM, Crawley JN, Sandhoff K, Suzuki K, Proia RL.

Proc Natl Acad Sci U S A. 1997 Jul 22;94(15):8138-43.PMID: 9223328 [PubMed - indexed for MEDLINE]Free PMC ArticleFree textRelated citations

Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease.

Pelled D, Riebeling C, van Echten-Deckert G, Sandhoff K, Futerman AH.

Neuropathol Appl Neurobiol. 2003 Aug;29(4):341-9.PMID: 12887594 [PubMed - indexed for MEDLINE]Related citations

Structure and stability of internodal myelin in mouse models of hereditary neuropathy.

Avila RL, Inouye H, Baek RC, Yin X, Trapp BD, Feltri ML, Wrabetz L, Kirschner DA.

J Neuropathol Exp Neurol. 2005 Nov;64(11):976-90.PMID: 16254492 [PubMed - indexed for MEDLINE]Related citations

Development of infertility at young adult age in a mouse model of human Sandhoff disease.

Juneja SC.

Reprod Fertil Dev. 2002;14(7-8):407-12.PMID: 12617783 [PubMed - indexed for MEDLINE]Related citations

Imaging MALDI mass spectrometry using an oscillating capillary nebulizer matrix coating system and its application to analysis of lipids in brain from a mouse model of Tay-Sachs/Sandhoff disease.

Chen Y, Allegood J, Liu Y, Wang E, Cachón-Gonzalez B, Cox TM, Merrill AH Jr, Sullards MC.

Anal Chem. 2008 Apr 15;80(8):2780-8. Epub 2008 Mar 4.PMID: 18314967 [PubMed - indexed for MEDLINE]Related citations

Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy.

Ramakrishnan H, Hedayati KK, Lüllmann-Rauch R, Wessig C, Fewou SN, Maier H, Goebel HH, Gieselmann V, Eckhardt M.

J Neurosci. 2007 Aug 29;27(35):9482-90.PMID: 17728461 [PubMed - indexed for MEDLINE]Free ArticleRelated citations

Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.

Pelled D, Lloyd-Evans E, Riebeling C, Jeyakumar M, Platt FM, Futerman AH.

J Biol Chem. 2003 Aug 8;278(32):29496-501. Epub 2003 May 19.PMID: 12756243 [PubMed - indexed for MEDLINE]Free ArticleRelated citations

Plasmid-based gene transfer ameliorates visceral storage in a mouse model of Sandhoff disease.

Yamaguchi A, Katsuyama K, Suzuki K, Kosaka K, Aoki I, Yamanaka S.

J Mol Med. 2003 Mar;81(3):185-93. Epub 2003 Feb 12.PMID: 12682727 [PubMed - indexed for MEDLINE]Related citations

Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases.

Phaneuf D, Wakamatsu N, Huang JQ, Borowski A, Peterson AC, Fortunato SR, Ritter G, Igdoura SA, Morales CR, Benoit G, Akerman BR, Leclerc D, Hanai N, Marth JD, Trasler JM, Gravel RA.

Hum Mol Genet. 1996 Jan;5(1):1-14.PMID: 8789434 [PubMed - indexed for MEDLINE]Free ArticleRelated citations

Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis.

Sango K, McDonald MP, Crawley JN, Mack ML, Tifft CJ, Skop E, Starr CM, Hoffmann A, Sandhoff K, Suzuki K, Proia RL.

Nat Genet. 1996 Nov;14(3):348-52.PMID: 8896570 [PubMed - indexed for MEDLINE]Related citations

Mechanism of abnormal growth in astrocytes derived from a mouse model of GM2 gangliosidosis.

Kawashima N, Tsuji D, Okuda T, Itoh K, Nakayama K.

J Neurochem. 2009 Nov;111(4):1031-41. Epub 2009 Sep 18.PMID: 19765188 [PubMed - indexed for MEDLINE]Related citations

I. Abnormalities in cells of the testis, efferent ducts, and epididymis in juvenile and adult mice with beta-hexosaminidase A and B deficiency.

Adamali HI, Somani IH, Huang JQ, Mahuran D, Gravel RA, Trasler JM, Hermo L.

J Androl. 1999 Nov-Dec;20(6):779-802.PMID: 10591618 [PubMed - indexed for MEDLINE]Free ArticleRelated citations

X-ray diffraction study of myelin structure in immature and mutant mice.

Kirschner DA, Sidman RL.

Biochim Biophys Acta. 1976 Sep 21;448(1):73-87.PMID: 971429 [PubMed - indexed for MEDLINE]Related citations