Determinants of NPC1 expression and action: Key promoter regions, posttranscriptional control, and the importance of a 'cysteine-rich' loop

Experimental Cell Research

Volumn 259, Issue 1, 2000, Pages 247-256

  Watari, Hidemichi ^a   Blanchette Mackie, E Joan ^b   Dwyer, Nancy K ^b   Watari, Michiko ^a   Burd, Christopher G ^a   Patel, Shutish ^c   Pentchev, Peter G ^d   Strauss III, Jerome F ^a  

^a HOSPITAL OF THE UNIVERSITY OF PENNSYLVANIA   (United States)

^b NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHOLESTEROL; CIS ACTING ELEMENT; CYSTEINE; GLYCOPROTEIN; ISOLEUCINE; MESSENGER RNA; NIEMANN PICK C 1 PROTEIN; PROGESTERONE; RECOMBINANT PROTEIN; THREONINE; UNCLASSIFIED DRUG; ZINC;

AMINO ACID SUBSTITUTION; ARTICLE; CHOLESTEROL TRANSPORT; CONTROLLED STUDY; GENE DELETION; GENE FUNCTION; GRANULOSA CELL; HUMAN; HUMAN CELL; LYSOSOME; METAL BINDING; NIEMANN PICK DISEASE; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN EXPRESSION; PROTEIN PROCESSING; PROTEIN SECONDARY STRUCTURE; STRUCTURE ACTIVITY RELATION; STRUCTURE ANALYSIS; TRANSCRIPTION REGULATION;

CRICETINAE; CRICETULUS GRISEUS;

EID: 0034714439     PISSN: 00144827     EISSN: None     Source Type: Journal    
DOI: 10.1006/excr.2000.4976     Document Type: Article

References (36)

1. Niemann-Pick C1 gene: Homology to mediators of cholesterol homeostasis
2. Mutations in NPC1 highlight a conserved NPC1-specific cyteine-rich domain
3. Niemann-Pick C1 disease: The I1061T substitution is a frequent mutant allele in patients of western European descent and correlates with a classical juvenile phenotype
4. Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-golgi network
5. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo
6. Cholesterol distribution in Golgi, lysosomes and endoplasmic reticulum
7. Niemann-Pick disease type C
8. 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
9. Characterization of Chinese-hamster ovary cells that are resistant to 3β-[2-(diethylamino)ethoxy]androst-5-en-17-one inhibition of low-density lipoprotein-derived cholesterol metabolism
10. The intracellular transport of low-density lipoprotein derived cholesterol is inhibited in Chinese-hamster ovary cells cultured with 3β-[2-(diethylamino)ethoxy]androst-5-en-17-one
11. Intracellular cholesterol transport
12. Quantitative analysis of hydrophobic amine inhibition of intracellular cholesterol transport
13. Cholesterol movement in Niemann-Pick type C cells and in cells treated with amphiphiles
14. NPC1-containing compartment of human granulosa-lutein cells: A role in the intracellular trafficking of cholesterol supporting steroidogenesis
15. Localization of Niemann-Pick C1 protein in astrocytes: Implications for neuronal degeneration in Niemann-Pick type C disease
16. Murine model of Niemann-Pick C disease: Mutation in a cholesterol homeostasis gene
17. Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization
18. Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein
19. Topological analysis of NPC1 reveals that the membrane orientation of the putative sterol sensing domain is identical to those of HMG CoA reductase and SCAP
20. The genomic organization and polymorphism analysis of the human Niemann-Pick C1 gene
21. Proliferating human granulosa-lutein cells in long term monolayer culture: Expression of aromatase, cholesterol side-chain cleavage, and 3β hydroxysteroid dehydrogenase
22. Pure, post-mitotic, polarized human neurons derived from Ntera 2 cells provide a system for expressing exogenous proteins in terminally differentiated neurons
23. Isolation and characterization of chinese hamster ovary cell mutants defective in intracellular low-density lipoprotein cholesterol trafficking
24. Chinese hamster ovary cell mutants affecting cholesterol metabolism
25. Role of the Niemann-Pick type C1 protein in intracellular trafficking of low density lipoprotein-derived cholesterol
26. The RING finger - A novel protein sequence motif related to the zinc-finger
27. Phorbol ester binding to protein kinase C requires a cysteine-rich zinc-finger-like sequence
28. Phosphoinositide 3-kinases and their FYVE domain-containing effectors as regulators of vacuolar/lysosomal membrane trafficking pathways
29. Removal of poly(A) and consequent degradation of c-fos messenger-RNA facilitated by 3' AU-rich sequences
30. Protein synthesis inhibitors differentially superinduce c-fos and c-jun by 3 distinct mechanisms. Lack of evidence for labile repressors
31. Regulation of the lysosome-associated membrane protein in a sucrose model of lysosomal storage
32. The role of lipoproteins in steroidogenesis and cholesterol metabolism in steroidogenic glands
33. Human follicular fluid vascular endothelial growth factor concentrations are correlated with luteinization in spontaneously developing follicles
34. Luteinization: Models and markers
35. Luteinization and gonadotrophins up-regulate the Niemann-Pick gene in the porcine ovary
36. Late endosomal membranes rich in lysobiphosphatidic acid regulate cholesterol transport