PMID- 30738290
OWN - NLM
STAT- MEDLINE
DCOM- 20190705
LR  - 20201209
IS  - 1878-1705 (Electronic)
IS  - 1567-5769 (Linking)
VI  - 69
DP  - 2019 Apr
TI  - Morphological and functional analysis of beige (Chediak-Higashi syndrome) mouse 
      mast cells with giant granules.
PG  - 202-212
LID - S1567-5769(19)30119-5 [pii]
LID - 10.1016/j.intimp.2019.01.053 [doi]
AB  - Chediak-Higashi syndrome is a rare autosomal recessive disease that causes 
      hypopigmentation, recurrent infections, mild coagulation defects and neurological 
      problems. Beige mice carry a mutation in the lysosome trafficking regulator 
      (LYST) gene and display some of the key characteristics of human Chediak-Higashi 
      syndrome, in particular, a high susceptibility to infection due to aberrant 
      natural killer (NK) cell and polymorphonuclear leucocyte function. Morphological 
      analysis of beige mice reveals the presence of enlarged lysosomes in a variety of 
      cell types, including leucocytes, hepatocytes, fibroblasts and renal tubule 
      cells. To examine the process of granule maturation and degranulation in beige 
      mice mast cells, morphological studies have been conducted using a combination of 
      electrophysiological techniques; however, few functional studies have been 
      conducted with mast cells, such as mediator release. The aim of the present study 
      was to determine the morphological and functional characteristics of skin and 
      peritoneal mast cells and bone marrow-derived mast cells of homozygous (bg/bg) 
      and heterozygous (bg/+) beige mice and wild-type (+/+) mice. The histamine 
      concentration was lower in the peritoneal and bone marrow-derived mast cells of 
      bg/bg mice compared with those of bg/+ and +/+ mice, but the histamine release 
      response was potentiated. In vivo studies of passive cutaneous anaphylaxis showed 
      no differences between bg/bg mice and either bg/+ or +/+ mice. Although bg/bg 
      mast cells with enlarged granules display specific exocytotic processes in vitro, 
      the consequences of mast cell activation in beige mice were similar to those of 
      wild-type mice in vivo.
CI  - Copyright (c) 2019 Elsevier B.V. All rights reserved.
FAU - Kiyoi, Takeshi
AU  - Kiyoi T
AD  - Division of Analytical Bio-medicine, Advanced Research Support Center, Ehime 
      University, Shitsukawa, Toon, Ehime 791-0295, Japan; Department of Pharmacology, 
      Ehime University Graduate School of Medicine Shitsukawa, Toon, Ehime 791-0295, 
      Japan.
FAU - Liu, Shuang
AU  - Liu S
AD  - Department of Pharmacology, Ehime University Graduate School of Medicine 
      Shitsukawa, Toon, Ehime 791-0295, Japan.
FAU - Sahid, Muhammad Novrizal Abdi
AU  - Sahid MNA
AD  - Department of Pharmacology, Ehime University Graduate School of Medicine 
      Shitsukawa, Toon, Ehime 791-0295, Japan.
FAU - Shudou, Masachika
AU  - Shudou M
AD  - Division of Analytical Bio-medicine, Advanced Research Support Center, Ehime 
      University, Shitsukawa, Toon, Ehime 791-0295, Japan.
FAU - Ogasawara, Masahito
AU  - Ogasawara M
AD  - Department of Pathogenesis and Control of Oral Diseases, Division of Dental 
      Pharmacology, Iwate Medical University School of Dentistry, Morioka, Iwate 
      020-8505, Japan.
FAU - Mogi, Masaki
AU  - Mogi M
AD  - Department of Pharmacology, Ehime University Graduate School of Medicine 
      Shitsukawa, Toon, Ehime 791-0295, Japan. Electronic address: 
      mmogi@m.ehime-u.ac.jp.
FAU - Maeyama, Kazutaka
AU  - Maeyama K
AD  - Department of Pharmacology, Ehime University Graduate School of Medicine 
      Shitsukawa, Toon, Ehime 791-0295, Japan.
LA  - eng
PT  - Journal Article
DEP - 20190206
PL  - Netherlands
TA  - Int Immunopharmacol
JT  - International immunopharmacology
JID - 100965259
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (Lyst protein, mouse)
RN  - 0 (Proteins)
RN  - 0 (Vesicular Transport Proteins)
RN  - 820484N8I3 (Histamine)
SB  - IM
MH  - Animals
MH  - Cell Degranulation
MH  - Cells, Cultured
MH  - Chediak-Higashi Syndrome/genetics/*immunology
MH  - Cytoplasmic Granules/*pathology
MH  - Disease Models, Animal
MH  - Histamine/metabolism
MH  - Homozygote
MH  - Humans
MH  - Intracellular Signaling Peptides and Proteins
MH  - Killer Cells, Natural/*immunology
MH  - Lysosomes/*pathology
MH  - Mast Cells/pathology/*physiology
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Mutant Strains
MH  - Mutation/genetics
MH  - Neutrophils/*immunology
MH  - Proteins/genetics
MH  - Vesicular Transport Proteins
EDAT- 2019/02/10 06:00
MHDA- 2019/07/06 06:00
CRDT- 2019/02/10 06:00
PHST- 2019/01/18 00:00 [received]
PHST- 2019/01/30 00:00 [accepted]
PHST- 2019/02/10 06:00 [pubmed]
PHST- 2019/07/06 06:00 [medline]
PHST- 2019/02/10 06:00 [entrez]
AID - S1567-5769(19)30119-5 [pii]
AID - 10.1016/j.intimp.2019.01.053 [doi]
PST - ppublish
SO  - Int Immunopharmacol. 2019 Apr;69:202-212. doi: 10.1016/j.intimp.2019.01.053. Epub 
      2019 Feb 6.