(Q36736837)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

title

In vivo antisense oligonucleotide reduction of NPC1 expression as a novel mouse model for Niemann Pick type C- associated liver disease (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

4

1 reference

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6 January 2020

author name string

Victoria M Rimkunas

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

Mark J Graham

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

Rosanne M Crooke

series ordinal

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

language of work or name

English

0 references

publication date

1 May 2008

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

volume

47

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

issue

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

page(s)

1504-1512

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

Clues to neuro-degeneration in Niemann-Pick type C disease from global gene expression profiling

6 January 2020

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Lipid imbalance in the neurological disorder, Niemann-Pick C disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

NPC2 is expressed in human and murine liver and secreted into bile: potential implications for body cholesterol homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

The niemann-pick disease genes; regulators of cellular cholesterol homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

A simple method for the isolation and purification of total lipides from animal tissues

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Niemann-Pick disease type C.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Pharmacokinetic properties of 2'-O-(2-methoxyethyl)-modified oligonucleotide analogs in rats

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Cholesterol accumulation in tissues of the Niemann-pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Genetic heterogeneity in Niemann-Pick C disease: a study using somatic cell hybridization and linkage analysis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Role of liver in the maintenance of cholesterol and low density lipoprotein homeostasis in different animal species, including humans.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Hepatic fibrosis caused by alcohol

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Liver failure and the sea-blue histiocyte/adult Niemann-Pick disease. Case report and review of the literature

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

An apolipoprotein B antisense oligonucleotide lowers LDL cholesterol in hyperlipidemic mice without causing hepatic steatosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Niemann-Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease.

28 June 2018

1 reference

Niemann-pick disease type C in neonatal cholestasis at a North American Center

21 January 2018

1 reference

Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease

21 January 2018

1 reference

Liver disease with altered bile acid transport in Niemann-Pick C mice on a high-fat, 1% cholesterol diet

21 January 2018

1 reference

Rescue of neurodegeneration in Niemann-Pick C mice by a prion-promoter-driven Npc1 cDNA transgene.

21 January 2018

1 reference

Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption

21 January 2018

1 reference

Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.

21 January 2018

1 reference

Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease

21 January 2018

1 reference

Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Identification of unusual 7-oxygenated bile acid sulfates in a patient with Niemann-Pick disease, type C.

5 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2440313

Differential Regulation of ATP Binding Cassette Protein A1 Expression and ApoA-I Lipidation by Niemann-Pick Type C1 in Murine Hepatocytes and Macrophages

5 September 2018

1 reference

Lipid homeostasis and lipoprotein secretion in Niemann-Pick C1-deficient hepatocytes

21 January 2018

1 reference

12 December 2020

Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease

1 reference

12 December 2020

Characterization of liver disease and lipid metabolism in the Niemann-Pick C1 mouse

1 reference

12 December 2020

Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse

1 reference

12 December 2020

Centripetal cholesterol flow from the extrahepatic organs through the liver is normal in mice with mutated Niemann-Pick type C protein (NPC1)

1 reference

12 December 2020

Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts

1 reference

12 December 2020

Progressive neonatal liver failure due to type C Niemann-Pick disease

1 reference

12 December 2020

Fetal Niemann-Pick disease type C: ultrastructural and lipid findings in liver and spleen

1 reference

12 December 2020

Severe failure to thrive and liver dysfunction as the main manifestations of a new variant of Niemann-Pick disease

1 reference

12 December 2020

Niemann-Pick disease type C presenting as neonatal hepatitis: report of one case

1 reference

12 December 2020

Progressive liver failure in a patient with adult Niemann-Pick disease associated with generalized AL amyloidosis

1 reference

12 December 2020

Identifiers

DOI

10.1002/HEP.22327

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18438776%20AND%20SRC:MED&resulttype=core&format=json

6 January 2020

PubMed publication ID

18438776

1 reference