PMID- 10559883
OWN - NLM
STAT- MEDLINE
DCOM- 19991216
LR  - 20131121
IS  - 1465-7392 (Print)
IS  - 1465-7392 (Linking)
VI  - 1
IP  - 2
DP  - 1999 Jun
TI  - Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol
      transport.
PG  - 113-8
AB  - The fate of free cholesterol released after endocytosis of low-density
      lipoproteins remains obscure. Here we report that late endosomes have a pivotal
      role in intracellular cholesterol transport. We find that in the genetic disease 
      Niemann-Pick type C (NPC), and in drug-treated cells that mimic NPC, cholesterol 
      accumulates in late endosomes and sorting of the lysosomal enzyme receptor is
      impaired. Our results show that the characteristic network of lysobisphosphatidic
      acid-rich membranes contained within multivesicular late endosomes regulates
      cholesterol transport, presumably by acting as a collection and distribution
      device. The results also suggest that similar endosomal defects accompany the
      anti-phospholipid syndrome and NPC.
FAU - Kobayashi, T
AU  - Kobayashi T
AD  - Department of Biochemistry, University of Geneva, Switzerland.
FAU - Beuchat, M H
AU  - Beuchat MH
FAU - Lindsay, M
AU  - Lindsay M
FAU - Frias, S
AU  - Frias S
FAU - Palmiter, R D
AU  - Palmiter RD
FAU - Sakuraba, H
AU  - Sakuraba H
FAU - Parton, R G
AU  - Parton RG
FAU - Gruenberg, J
AU  - Gruenberg J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Nat Cell Biol
JT  - Nature cell biology
JID - 100890575
RN  - 0 (Lysophospholipids)
RN  - 0 (Membrane Lipids)
RN  - 0 (Monoglycerides)
RN  - 0 (Receptor, IGF Type 2)
RN  - 0 (Recombinant Proteins)
RN  - 0 (bis(monoacylglyceryl)phosphate)
RN  - 97C5T2UQ7J (Cholesterol)
RN  - J41CSQ7QDS (Zinc)
SB  - IM
CIN - Nat Cell Biol. 1999 Jun;1(2):E37-8. PMID: 10559892
MH  - Animals
MH  - Antiphospholipid Syndrome/genetics/metabolism
MH  - Cell Line
MH  - Cells, Cultured
MH  - Cholesterol/*metabolism
MH  - Cricetinae
MH  - Endocytosis
MH  - Endosomes/drug effects/genetics/*metabolism
MH  - Fibroblasts/metabolism/ultrastructure
MH  - Humans
MH  - Intracellular Membranes/*metabolism
MH  - Lysophospholipids/*metabolism
MH  - Lysosomes/metabolism
MH  - Membrane Lipids/*metabolism
MH  - Monoglycerides
MH  - Niemann-Pick Diseases/genetics/*metabolism/pathology
MH  - Receptor, IGF Type 2/metabolism
MH  - Recombinant Proteins/metabolism
MH  - Skin/*metabolism/pathology/ultrastructure
MH  - Zinc/pharmacology
EDAT- 1999/11/13 09:00
MHDA- 2001/03/23 10:01
CRDT- 1999/11/13 09:00
PHST- 1999/11/13 09:00 [pubmed]
PHST- 2001/03/23 10:01 [medline]
PHST- 1999/11/13 09:00 [entrez]
AID - 10.1038/10084 [doi]
PST - ppublish
SO  - Nat Cell Biol. 1999 Jun;1(2):113-8. doi: 10.1038/10084.