• Publications
  • Influence
COPII and the regulation of protein sorting in mammals
Secretory proteins are transported to the Golgi complex in vesicles that bud from the endoplasmic reticulum. The cytoplasmic coat protein complex II (COPII) is responsible for cargo sorting andExpand
  • 218
  • 16
COPII and the regulation of protein sorting in mammals
Nat. Cell Biol. 14, 20–28 (2012); published online 22 December 2011; corrected after print 6 January 2012 In the version of this review initially published online and in print, the key in figure 2Expand
  • 70
  • 10
Structure/Function Analysis of Four Core ESCRT‐III Proteins Reveals Common Regulatory Role for Extreme C‐Terminal Domain
Endosomal sorting complex required for transport‐III (ESCRT‐III) is a large complex built from related ESCRT‐III proteins involved in multivesicular body biogenesis. Little is known about theExpand
  • 166
  • 9
Novel interactions of ESCRT-III with LIP5 and VPS4 and their implications for ESCRT-III disassembly.
The AAA+ ATPase VPS4 plays an essential role in multivesicular body biogenesis and is thought to act by disassembling ESCRT-III complexes. VPS4 oligomerization and ATPase activity are promoted byExpand
  • 73
  • 6
  • PDF
Structure of cellular ESCRT-III spirals and their relationship to HIV budding
The ESCRT machinery along with the AAA+ ATPase Vps4 drive membrane scission for trafficking into multivesicular bodies in the endocytic pathway and for the topologically related processes of viralExpand
  • 90
  • 5
  • PDF
CHMP4B, a novel gene for autosomal dominant cataracts linked to chromosome 20q.
Cataracts are a clinically diverse and genetically heterogeneous disorder of the crystalline lens and a leading cause of visual impairment. Here we report linkage of autosomal dominant "progressiveExpand
  • 84
  • 3
Cell biology of the ESCRT machinery.
The ESCRT (endosomal sorting complex required for transport) machinery comprises a set of protein complexes that regulate sorting and trafficking into multivesicular bodies en route to the lysosome.Expand
  • 88
  • 2
Mouse model of N-acetylgalactosamine-6-sulfate sulfatase deficiency (Galns-/-) produced by targeted disruption of the gene defective in Morquio A disease.
Mucopolysaccharidosis IVA is an autosomal recessive disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS), a lysosomal enzyme required for the stepwise degradation ofExpand
  • 67
  • 1