Animal models of lysosomal disease: an overview (Q38578684)

4 September 2017

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title

Animal models of lysosomal disease: an overview (English)

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4 September 2017

Suzuki K

1

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PubMed ID

9728334

4 September 2017

Månsson JE

series ordinal

2

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4 September 2017

1 August 1998

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Journal of Inherited Metabolic Disease

4 September 2017

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4 September 2017

21

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4 September 2017

5

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4 September 2017

540-547

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https://scigraph.springernature.com/pub.10.1023/a:1005466905180

4 September 2017

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cites work

Mouse model for the lysosomal disorder galactosialidosis and correction of the phenotype with overexpressing erythroid precursor cells

1 reference

12 December 2020

Mice deficient in lysosomal acid phosphatase develop lysosomal storage in the kidney and central nervous system

1 reference

12 December 2020

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

1 reference

12 December 2020

Donor-derived cells in the central nervous system of twitcher mice after bone marrow transplantation.

1 reference

12 December 2020

Generalized CNS disease and massive GM1-ganglioside accumulation in mice defective in lysosomal acid beta-galactosidase

1 reference

12 December 2020

Lactosylceramide beta-galactosidase in human sphingolipidoses. Evidence for two genetically distinct enzymes

1 reference

12 December 2020

Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin

1 reference

12 December 2020

Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphi

1 reference

12 December 2020

Targeted gene replacement.

1 reference

12 December 2020

Effect of bone marrow transplantation on enzyme levels and clinical course in the neurologically affected twitcher mouse

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12 December 2020

Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse.

1 reference

12 December 2020

Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease

1 reference

12 December 2020

Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosides

1 reference

12 December 2020

Mice deficient in all forms of lysosomal beta-hexosaminidase show mucopolysaccharidosis-like pathology

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12 December 2020

Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease).

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12 December 2020

Characterization of the rhesus monkey galactocerebrosidase (GALC) cDNA and gene and identification of the mutation causing globoid cell leukodystrophy (Krabbe disease) in this primate

1 reference

12 December 2020

Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins

1 reference

12 December 2020

Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease

1 reference

12 December 2020

Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy

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12 December 2020

Differential sorting of lysosomal enzymes in mannose 6-phosphate receptor-deficient fibroblasts

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12 December 2020

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

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12 December 2020

Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.

1 reference

12 December 2020

Murine mucopolysaccharidosis type VII: long term therapeutic effects of enzyme replacement and enzyme replacement followed by bone marrow transplantation

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12 December 2020

The twitcher mouse: a model for Krabbe disease and for experimental therapies

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12 December 2020

Neurological manifestations of knockout mice with beta-galactosidase deficiency

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12 December 2020

Hematopoietic cell transplantation in the twitcher mouse. The effects of pretransplant conditioning with graded doses of busulfan.

1 reference

12 December 2020

The Two Mannose 6-Phosphate Receptors Transport Distinct Complements of Lysosomal Proteins

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12 December 2020

Correction of murine mucopolysaccharidosis VII by a human beta-glucuronidase transgene

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12 December 2020

Disruption of murine Hexa gene leads to enzymatic deficiency and to neuronal lysosomal storage, similar to that observed in Tay-Sachs disease

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12 December 2020

Mouse models of human lysosomal diseases.

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12 December 2020

Targeted disruption of the mouse cation-dependent mannose 6-phosphate receptor results in partial missorting of multiple lysosomal enzymes.

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12 December 2020

Substrate specificities of the two genetically distinct human brain β-galactosidases

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12 December 2020

Identifiers

DOI

10.1023/A:1005466905180

1 reference

PubMed ID

9728334

Dimensions Publication ID

4 September 2017

0 references

1 reference

PubMed ID

9728334