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Alternative mechanisms for trafficking of lysosomal enzymes in mannose 6-phosphate receptor-deficient mice are cell type-specific.
TLDR
Surprisingly, the ability of MPR-deficient cells to transport newly synthesized lysosomal enzymes to lYSosomes and the underlying mechanisms were found to depend on the cell type, and recapture by hepatocytes is remarkably effective in vivo and can result in lysOSomal enzyme levels even above normal.
I-cell disease-like phenotype in mice deficient in mannose 6-phosphate receptors
TLDR
Triple deficient mice surviving the first postnatal day had normal viability and developed a phenotype resembling human I-cell disease, and 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.
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