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I-cell disease-like phenotype in mice deficient in mannose 6-phosphate receptors

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Transgenic Research : Volume 7, Issue 1, January 1998

Transgenic Research : Volume 6

I-cell disease-like phenotype in mice deficient in mannose 6-phosphate receptors

Content Provider	Springer Nature Link
Author	Dittmer, Frank Hafner, Angela Ulbrich, Eva J. Moritz, Jörg D. Schmidt, Peter Schmahl, Wolfgang Pohlmann, Regina Figura, Kurt V.
Copyright Year	1998
Abstract	Mannose 6-phosphate receptor deficient mice were generated by crossing mice carrying null alleles for Igf2 and the 300 kDa and 46 kDa mannose 6-phosphate receptors, Mpr300 and Mpr46. Pre- and perinatal lethality of mice nullizygous for Igf2, Mpr300 and Mpr46 was increased. Triple deficient mice surviving the first postnatal day had normal viability and developed a phenotype resembling human I-cell disease. The triple deficient mice were characterized by dwarfism, facial dysplasia, waddling gait, dysostosis multiplex, elevated lysosomal enzymes in serum and histological signs of lysosomal storage predominantly in fibroblasts, but also in parenchymal cells of brain and liver. A paternally inherited Mpr300 wild type allele that is normally inactive in mice due to imprinting was reactivated in some tissues of mice lacking IGF II and MPR 46 and carrying a maternal Mpr300 null allele. Inspite of the partial reactivation the phenotype of these mice was similar to that of triple deficient mice.
Starting Page	473
Ending Page	483
Page Count	11
File Format	PDF
ISSN	09628819
Journal	Transgenic Research
Volume Number	7
Issue Number	6
e-ISSN	15739368
Language	English
Publisher	Kluwer Academic Publishers
Publisher Date	1998-01-01
Publisher Place	Dordrecht
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Human Genetics Plant Sciences Animal Anatomy / Morphology / Histology
Content Type	Text
Resource Type	Article
Subject	Genetics Animal Science and Zoology Biotechnology Agronomy and Crop Science

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