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(Received for publication, September 3, 1997)
From the Department of Pathology and the § Department of
Medicine, University of Utah, Salt Lake City, Utah 84132
The human autosomal recessive disorder
Chediak-Higashi syndrome and its murine homologue beige
are associated with the formation of giant lysosomes that cluster near
the perinuclear region of cells. We prepared a polyclonal antiserum
against a glutathione S-transferase-Beige fusion protein
and demonstrated by Western analysis that the beige gene
encodes a protein of 400 kDa that is expressed in cultured murine
fibroblasts as well as most mouse tissues. The protein was not detected
in either cultured fibroblasts or mouse tissues from two different
beige mutants. Cultured fibroblasts transformed with
multiple copies of yeast artificial chromosomes that contain the
full-length beige gene showed much higher levels of Beige
protein than either wild type fibroblasts or mouse tissues. Subcellular
fractionation experiments demonstrated that the Beige protein was
cytosolic and, under the conditions of isolation, had no measurable
membrane association. Cultured mouse fibroblasts in which the Beige
protein was overexpressed had smaller than normal lysosomes that were
more peripherally distributed than in control cells. These findings,
coupled with earlier published results, suggest that the Beige protein
regulates lysosomal fission.
Volume 272, Number 47,
Issue of November 21, 1997
pp. 29790-29794
©1997 by The American Society for Biochemistry and Molecular Biology, Inc.
The Beige/Chediak-Higashi Syndrome Gene Encodes a Widely
Expressed Cytosolic Protein
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