PubMed

Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene.

Franken S, Wittke D, Mansson JE, D'Hooge R, De Deyn PP, Lüllmann-Rauch R, Matzner U, Gieselmann V.

Lipids Health Dis. 2006 Aug 7;5:21.PMID: 16893448 [PubMed - indexed for MEDLINE]Related articlesFree article

Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy.

Eckhardt M, Hedayati KK, Pitsch J, Lüllmann-Rauch R, Beck H, Fewou SN, Gieselmann V.

J Neurosci. 2007 Aug 22;27(34):9009-21.PMID: 17715338 [PubMed - indexed for MEDLINE]Related articlesFree article

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]Related articlesFree article

Lysosomal sulfatide storage in the brain of arylsulfatase A-deficient mice: cellular alterations and topographic distribution.

Wittke D, Hartmann D, Gieselmann V, Lüllmann-Rauch R.

Acta Neuropathol. 2004 Oct;108(4):261-71. Epub 2004 Aug 20.PMID: 15322834 [PubMed - indexed for MEDLINE]Related articles

Oligodendrocyte-specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT-deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level.

Zöller I, Büssow H, Gieselmann V, Eckhardt M.

Glia. 2005 Nov 15;52(3):190-8.PMID: 15968630 [PubMed - indexed for MEDLINE]Related articles

Morphological alterations in the inner ear of the arylsulfatase A-deficient mouse.

Coenen R, Gieselmann V, Lüllmann-Rauch R.

Acta Neuropathol. 2001 May;101(5):491-8.PMID: 11484821 [PubMed - indexed for MEDLINE]Related articles

Sulfatide storage in visceral organs of arylsulfatase A-deficient mice.

Schott I, Hartmann D, Gieselmann V, Lüllmann-Rauch R.

Virchows Arch. 2001 Jul;439(1):90-6.PMID: 11499846 [PubMed - indexed for MEDLINE]Related articles

A spontaneously immortalized Schwann cell line to study the molecular aspects of metachromatic leukodystrophy.

Saravanan K, Büssow H, Weiler N, Gieselmann V, Franken S.

J Neurosci Methods. 2007 Apr 15;161(2):223-33. Epub 2007 Jan 3.PMID: 17204333 [PubMed - indexed for MEDLINE]Related articles

Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy.

Matzner U, Breiden B, Schwarzmann G, Yaghootfam A, Fluharty AL, Hasilik A, Sandhoff K, Gieselmann V.

J Biol Chem. 2009 Apr 3;284(14):9372-81. Epub 2009 Feb 18.PMID: 19224915 [PubMed - indexed for MEDLINE]Related articles

Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations.

Matzner U, Hartmann D, Lüllmann-Rauch R, Coenen R, Rothert F, Månsson JE, Fredman P, D'Hooge R, De Deyn PP, Gieselmann V.

Gene Ther. 2002 Jan;9(1):53-63.PMID: 11850723 [PubMed - indexed for MEDLINE]Related articlesFree article

Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase.

Lüllmann-Rauch R, Matzner U, Franken S, Hartmann D, Gieselmann V.

Histochem Cell Biol. 2001 Aug;116(2):161-9.PMID: 11685544 [PubMed - indexed for MEDLINE]Related articles

Early signs of neurolipidosis-related behavioural alterations in a murine model of metachromatic leukodystrophy.

Stroobants S, Leroy T, Eckhardt M, Aerts JM, Berckmans D, D'Hooge R.

Behav Brain Res. 2008 Jun 3;189(2):306-16. Epub 2008 Jan 31.PMID: 18336930 [PubMed - indexed for MEDLINE]Related articles

Metachromatic leukodystrophy: molecular genetics and an animal model.

Gieselmann V, Matzner U, Hess B, Lüllmann-Rauch R, Coenen R, Hartmann D, D'Hooge R, DeDeyn P, Nagels G.

J Inherit Metab Dis. 1998 Aug;21(5):564-74. Review.PMID: 9728336 [PubMed - indexed for MEDLINE]Related articles

Accumulation of sulfatide in neuronal and glial cells of arylsulfatase A deficient mice.

Molander-Melin M, Pernber Z, Franken S, Gieselmann V, Månsson JE, Fredman P.

J Neurocytol. 2004 Jul;33(4):417-27.PMID: 15520527 [PubMed - indexed for MEDLINE]Related articles

Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy.

Matzner U, Herbst E, Hedayati KK, Lüllmann-Rauch R, Wessig C, Schröder S, Eistrup C, Möller C, Fogh J, Gieselmann V.

Hum Mol Genet. 2005 May 1;14(9):1139-52. Epub 2005 Mar 16.PMID: 15772092 [PubMed - indexed for MEDLINE]Related articlesFree article

Non-inhibitory antibodies impede lysosomal storage reduction during enzyme replacement therapy of a lysosomal storage disease.

Matzner U, Matthes F, Weigelt C, Andersson C, Eistrup C, Fogh J, Gieselmann V.

J Mol Med. 2008 Apr;86(4):433-42. Epub 2008 Mar 18.PMID: 18360747 [PubMed - indexed for MEDLINE]Related articles

Coexpression of formylglycine-generating enzyme is essential for synthesis and secretion of functional arylsulfatase A in a mouse model of metachromatic leukodystrophy.

Takakusaki Y, Hisayasu S, Hirai Y, Shimada T.

Hum Gene Ther. 2005 Aug;16(8):929-36.PMID: 16076251 [PubMed - indexed for MEDLINE]Related articles

Metachromatic leukodystrophy: recent research developments.

Gieselmann V.

J Child Neurol. 2003 Sep;18(9):591-4. Review.PMID: 14572136 [PubMed - indexed for MEDLINE]Related articles

Ex vivo cell-mediated gene therapy for metachromatic leukodystrophy using neurospheres.

Kawabata K, Migita M, Mochizuki H, Miyake K, Igarashi T, Fukunaga Y, Shimada T.

Brain Res. 2006 Jun 13;1094(1):13-23. Epub 2006 May 26.PMID: 16729983 [PubMed - indexed for MEDLINE]Related articles

Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide.

Ezoe T, Vanier MT, Oya Y, Popko B, Tohyama J, Matsuda J, Suzuki K, Suzuki K.

J Neurosci Res. 2000 Jan 15;59(2):170-8.PMID: 10650875 [PubMed - indexed for MEDLINE]Related articles