The therapeutic potential of pharmacological chaperones and proteosomal inhibitors, Celastrol and MG132 in the treatment of sialidosis (Q39296453)

10 September 2017

The therapeutic potential of pharmacological chaperones and proteosomal inhibitors, Celastrol and MG132 in the treatment of sialidosis (English)

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10 September 2017

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Erin M O'Leary

1

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PubMed ID

22898113

10 September 2017

Suleiman A Igdoura

series ordinal

2

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10 September 2017

24 July 2012

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Molecular Genetics and Metabolism

10 September 2017

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10 September 2017

107

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10 September 2017

1-2

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10 September 2017

173-185

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Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis

10 September 2017

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Mucolipidosis I: increased sialic acid content and deficiency of an alpha-N-acetylneuraminidase in cultured fibroblasts

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Vacuolization and alterations of lysosomal membrane proteins in cochlear marginal cells contribute to hearing loss in neuraminidase 1-deficient mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

The cherry-red spot-myoclonus syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Isolated acid neuraminidase deficiency: A distinct lysosomal storage disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Neuraminidase deficiency presenting as non-immune hydrops fetalis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Neuraminidase deficiency presenting as a nephrosialidosis: the first case detected in Poland

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Cloning and characterization of NEU2, a human gene homologous to rodent soluble sialidases.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Identification and expression of NEU3, a novel human sialidase associated to the plasma membrane

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Molecular cloning and characterization of NEU4, the fourth member of the human sialidase gene family

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Evidence for mitochondrial localization of a novel human sialidase (NEU4).

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Characterization of the sialidase molecular defects in sialidosis patients suggests the structural organization of the lysosomal multienzyme complex

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Sialidases in vertebrates: a family of enzymes tailored for several cell functions

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Sequence and structural analysis of the Asp-box motif and Asp-box beta-propellers; a widespread propeller-type characteristic of the Vps10 domain family and several glycoside hydrolase families

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Neu1 desialylation of sialyl alpha-2,3-linked beta-galactosyl residues of TOLL-like receptor 4 is essential for receptor activation and cellular signaling

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Lysosomal sialidase (neuraminidase-1) is targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assembly

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Intracellular distribution of lysosomal sialidase is controlled by the internalization signal in its cytoplasmic tail

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Neuraminidase-1 is required for the normal assembly of elastic fibers

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Lysosomal protective protein/cathepsin A. Role of the "linker" domain in catalytic activation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Mutations in sialidosis impair sialidase binding to the lysosomal multienzyme complex

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Molecular pathology of NEU1 gene in sialidosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Insight into substrate recognition and catalysis by the human neuraminidase 3 (NEU3) through molecular modeling and site-directed mutagenesis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Novel missense mutations in the human lysosomal sialidase gene in sialidosis patients and prediction of structural alterations of mutant enzymes

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Homology modeling of human sialidase enzymes NEU1, NEU3 and NEU4 based on the crystal structure of NEU2: hints for the design of selective NEU3 inhibitors.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Five novel mutations in the lysosomal sialidase gene (NEU1) in type II sialidosis patients and assessment of their impact on enzyme activity and intracellular targeting using adenovirus-mediated expression

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Chemical and biological approaches synergize to ameliorate protein-folding diseases.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Proteasome inhibitors: valuable new tools for cell biologists

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Comparative selectivity and specificity of the proteasome inhibitors BzLLLCOCHO, PS-341, and MG-132.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Proteasome inhibition leads to a heat-shock response, induction of endoplasmic reticulum chaperones, and thermotolerance

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Celastrol: Molecular targets of Thunder God Vine

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Celastrols as inducers of the heat shock response and cytoprotection.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

TNF-induced recruitment and activation of the IKK complex require Cdc37 and Hsp90

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Reduction of beta-amyloid pathology by celastrol in a transgenic mouse model of Alzheimer's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Cloning of the cDNA and gene encoding mouse lysosomal sialidase and correction of sialidase deficiency in human sialidosis and mouse SM/J fibroblasts

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Quantitative colocalization analysis of multicolor confocal immunofluorescence microscopy images: pushing pixels to explore biological phenomena

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Functional differences between two major ubiquitin receptors in the proteasome; S5a and hRpn13

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Recent development in mammalian sialidase molecular biology

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

A cellular perspective on conformational disease: the role of genetic background and proteostasis networks

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Current strategies in the management of lysosomal storage diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

New therapeutic options for lysosomal storage disorders: enzyme replacement, small molecules and gene therapy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Therapeutic approaches to protein-misfolding diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Therapeutic strategies to ameliorate lysosomal storage disorders--a focus on Gaucher disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Protective protein/cathepsin A rescues N-glycosylation defects in neuraminidase-1.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Biological and chemical approaches to diseases of proteostasis deficiency

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Inhibitors of the proteasome block the degradation of most cell proteins and the generation of peptides presented on MHC class I molecules

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

The ubiquitin ligase E6-AP is induced and recruited to aggresomes in response to proteasome inhibition and may be involved in the ubiquitination of Hsp70-bound misfolded proteins

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Celastrol can inhibit proteasome activity and upregulate the expression of heat shock protein genes, hsp30 and hsp70, in Xenopus laevis A6 cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Ganglioside GM3 sialidase activity in fibroblasts of normal individuals and of patients with sialidosis and mucolipidosis IV. Subcellular distribution and and some properties

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Oligosaccharide and Ganglioside Neuraminidase Activities of Mucolipidosis I (Sialidosis) and Mucolipidosis II (I-Cell Disease) Fibroblasts

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Secondary accumulation of gangliosides in lysosomal storage disorders

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Metabolism of GM1 ganglioside in cultured skin fibroblasts: anomalies in gangliosidoses, sialidoses, and sphingolipid activator protein (SAP, saposin) 1 and prosaposin deficient disorders

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Sialidase occurs in both membranes of the nuclear envelope and hydrolyzes endogenous GD1a

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Changes of the ganglioside pattern and content in human fibroblasts by high density cell population subculture progression.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Uptake and metabolism of exogenous glycosphingolipids by cultured cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Ganglioside loading of cultured fibroblasts: a provocative method for the diagnosis of the GM2 gangliosidoses.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Biosynthesis and degradation of mammalian glycosphingolipids

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Role of Neu4L sialidase and its substrate ganglioside GD3 in neuronal apoptosis induced by catechol metabolites

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

A new tumor-specific variant of GRP78 as target for antibody-based therapy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Endoproteolytic cleavage and proteasomal degradation of presenilin 2 in transfected cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Taking a bite: proteasomal protein processing

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Localized treatment with a novel FDA-approved proteasome inhibitor blocks the degradation of dystrophin and dystrophin-associated proteins in mdx mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

Lysosomal sialidase deficiency: increased ganglioside content in autopsy tissues of a sialidosis patient.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2012.07.013

inferred from DOI database lookup

7 January 2021

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Identifiers

DOI

10.1016/J.YMGME.2012.07.013

1 reference

10 September 2017

1 reference

PubMed ID

22898113