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Volume 54, Number 4 >

Lumenal localization in the endoplasmic reticulum of the C-terminal tail of an AE1mutant responsible for hereditary spherocytosis in cattle

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Please use this identifier to cite or link to this item:http://doi.org/10.14943/jjvr.54.4.191

Title: Lumenal localization in the endoplasmic reticulum of the C-terminal tail of an AE1mutant responsible for hereditary spherocytosis in cattle
Authors: Ito, Daisuke Browse this author
Otsuka, Yayoi Browse this author
Koshino, Ichiro Browse this author
Inaba, Mutsumi Browse this author →KAKEN DB
Keywords: AE1
endoplasmic reticulum
proteasome
Issue Date: 28-Feb-2007
Publisher: The Graduate School of Veterinary Medicine, Hokkaido University
Journal Title: Japanese Journal of Veterinary Research
Volume: 54
Issue: 4
Start Page: 191
End Page: 197
Abstract: An R664X nonsense mutant AE1 is responsible for dominant hereditary spherocytosis in cattle and is degraded by the proteasomal endoplasmic reticulum-associated degradation . The present study demonstrated that R664XAE1 translated in vitro had the trypsin-sensitve site identical to that of the wild-type AE1. The P661S/R664X mutant containing a possible N-glycosylation site at Asn660 showed an increase in size by 3 kDa both in the cellfree translation system and in transfected HEK293 cells. Moreover, steady state levels of R664X and P661S/R664X in HEK293 cells were markedly increased in the presence of a proteasome inhibitior. These findings indicate that the truncated C-terminal region of R664X AE1 has lumenal localization in the endoplasmic reticulum and is not accessible to proteasomal machineries in the cytosol.
Type: bulletin (article)
URI: http://hdl.handle.net/2115/20029
Appears in Collections:Japanese Journal of Veterinary Research > Volume 54, Number 4

Submitter: 獣医学部図書室

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