PMID- 22934826
OWN - NLM
STAT- MEDLINE
DCOM- 20130624
LR  - 20211021
IS  - 1600-0854 (Electronic)
IS  - 1398-9219 (Print)
IS  - 1398-9219 (Linking)
VI  - 13
IP  - 12
DP  - 2012 Dec
TI  - Drosophila mauve mutants reveal a role of LYST homologs late in the maturation of 
      phagosomes and autophagosomes.
PG  - 1680-92
LID - 10.1111/tra.12005 [doi]
AB  - Chediak-Higashi syndrome (CHS) is a lethal disease caused by mutations that 
      inactivate the lysosomal trafficking regulator protein (LYST). Patients suffer 
      from diverse symptoms including oculocutaneous albinism, recurrent infections, 
      neutropenia and progressive neurodegeneration. These defects have been traced 
      back to over-sized lysosomes and lysosome-related organelles (LROs) in different 
      cell types. Here, we explore mutants in the Drosophila mauve gene as a new model 
      system for CHS. The mauve gene (CG42863) encodes a large BEACH domain protein of 
      3535 amino acids similar to LYST. This reflects a functional homology between 
      these proteins as mauve mutants also display enlarged LROs, such as pigment 
      granules. This Drosophila model also replicates the enhanced susceptibility to 
      infections and we show a defect in the cellular immune response. Early stages of 
      phagocytosis proceed normally in mauve mutant hemocytes but, unlike in wild type, 
      late phagosomes fuse and generate large vacuoles containing many bacteria. 
      Autophagy is similarly affected in mauve fat bodies as starvation-induced 
      autophagosomes grow beyond their normal size. Together these data suggest a model 
      in which Mauve functions to restrict homotypic fusion of different pre-lysosomal 
      organelles and LROs.
CI  - (c) 2012 John Wiley & Sons A/S.
FAU - Rahman, Mokhlasur
AU  - Rahman M
AD  - Department of Neuroscience, UT Southwestern Medical Center, 5323 Harry Hines 
      Blvd, Dallas, TX 75390-9111, USA.
FAU - Haberman, Adam
AU  - Haberman A
FAU - Tracy, Charles
AU  - Tracy C
FAU - Ray, Sanchali
AU  - Ray S
FAU - Kramer, Helmut
AU  - Kramer H
LA  - eng
GR  - R01 EY010199/EY/NEI NIH HHS/United States
GR  - EY10199/EY/NEI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20120920
PL  - England
TA  - Traffic
JT  - Traffic (Copenhagen, Denmark)
JID - 100939340
RN  - 0 (Drosophila Proteins)
RN  - 0 (Vesicular Transport Proteins)
RN  - 0 (mv protein, Drosophila)
SB  - IM
MH  - Animals
MH  - Autophagy/genetics
MH  - Cytoplasmic Granules/metabolism
MH  - Drosophila/genetics/immunology/*metabolism
MH  - Drosophila Proteins/genetics/*metabolism
MH  - Escherichia coli
MH  - Hemocytes/*metabolism/microbiology/ultrastructure
MH  - Immunity, Innate/genetics
MH  - Lysosomes/metabolism
MH  - Mutation
MH  - Organelle Size/genetics
MH  - Phagocytosis/genetics
MH  - Phagosomes/*metabolism/ultrastructure
MH  - Vesicular Transport Proteins/genetics/*metabolism
PMC - PMC3528838
MID - NIHMS404252
EDAT- 2012/09/01 06:00
MHDA- 2013/06/26 06:00
PMCR- 2013/12/01
CRDT- 2012/09/01 06:00
PHST- 2012/01/31 00:00 [received]
PHST- 2012/08/28 00:00 [revised]
PHST- 2012/08/28 00:00 [accepted]
PHST- 2012/09/01 06:00 [entrez]
PHST- 2012/09/01 06:00 [pubmed]
PHST- 2013/06/26 06:00 [medline]
PHST- 2013/12/01 00:00 [pmc-release]
AID - 10.1111/tra.12005 [doi]
PST - ppublish
SO  - Traffic. 2012 Dec;13(12):1680-92. doi: 10.1111/tra.12005. Epub 2012 Sep 20.