• Publications
  • Influence
A transmembrane form of the prion protein in neurodegenerative disease.
TLDR
Aberrant regulation of protein biogenesis and topology at the endoplasmic reticulum can result in neurodegeneration. Expand
Transmissible and genetic prion diseases share a common pathway of neurodegeneration
TLDR
It is found that the effectiveness of accumulated PrPSc in causing neurodegenerative disease depends upon the predilection of host-encoded PrP to be made in the CtmPrP form. Expand
Rapid regulation of steroidogenesis by mitochondrial protein import
TLDR
The steroidogenic acute regulatory protein (StAR), a mitochondrial protein required for stress responses, reproduction, and sexual differentiation of male fetuses, exerts its activity transiently at the outer mitochondrial membrane rather than at its final resting place in the matrix. Expand
A multifunctional aqueous channel formed by CFTR.
TLDR
Oocytes expressing CFTR showed cAMP-stimulated transport of urea but not the larger solute sucrose, suggesting a pore-like aqueous pathway and providing functional evidence for water movement through an ion channel. Expand
Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis
TLDR
Biotinylation reveals a 32-kDa, covalently cross-linked S OD1-containing protein species produced not only in FALS caused by SOD1 mutation, but also in SALS, suggesting a common step in the pathogenesis between SALS and FALS. Expand
Membrane Protein Biogenesis: Regulated Complexity at the Endoplasmic Reticulum
TLDR
A handful of initial studies on complex substrates may suggest the functional regulation of protein biogenesis may be regulated by transcriptional and translational control as an additional means of generating diversity of gene expression. Expand
Regulation of protein biogenesis at the endoplasmic reticulum membrane.
TLDR
Each stage of protein biogenesis is examined as a potential site of regulation that could be exploited by the cell to effectively increase the diversity of functional gene expression. Expand
Regulation of protein topology by trans-acting factors at the endoplasmic reticulum.
TLDR
It is demonstrated here that the proper translocation of the prion protein (PrP), a substrate that can be synthesized in more than one topologic form, requires additional factors and, in the absence of these additional factors, PrP is synthesized exclusively in the transmembrane topology associated with the development of neurodegenerative disease. Expand
Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein
TLDR
Determinants in this unusual topogenic sequence that direct transmembrane topology are investigated, and it is demonstrated that a lumenally disposed charged domain is required for stop transfer at the adjacent hydrophobic domain, a precise spatial relationship between these domains is essential for efficient stop transfer. Expand
Evidence for an alternate model of human P-glycoprotein structure and biogenesis.
TLDR
It is concluded that the transmembrane topology of MDR1, an important member of the ATP binding cassette (ABC) transporter superfamily, is not as predicted and should be revised. Expand
...
1
2
3
4
5
...