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The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts.
Results indicate that either a regulation between ADAMs on the post-translational level or that other, not yet known, proteases are able to compensate for ADAM 10 deficiency, and points to the existence of tissue-specific 'teams' of different proteases exerting alpha-secretase activity.
μ1A‐adaptin‐deficient mice: lethality, loss of AP‐1 binding and rerouting of mannose 6‐phosphate receptors
The steady‐state distribution of the mannose 6‐phosphate receptors MPR46 and MPR300 in μ1A‐deficient cells is shifted to endosomes at the expense of the TGN, indicating that AP‐1 is required for retrograde endosome to TGN transport of the receptor.
Phosphorylation of the AP2 μ subunit by AAK1 mediates high affinity binding to membrane protein sorting signals
- D. Ricotta, S. Conner, S. Schmid, K. von Figura, S. Höning
- Biology, ChemistryJournal of Cell Biology
- 4 March 2002
It is proposed that phosphorylation of the AP2 μ2 subunit by AAK1 ensures high affinity binding of AP2 to sorting signals of cargo membrane proteins during the initial steps of receptor-mediated endocytosis.
A di‐leucine‐based motif in the cytoplasmic tail of LIMP‐II and tyrosinase mediates selective binding of AP‐3
Monitoring surface plasmon resonance observed that AP‐3 is able to interact with the tail of the lysosomal integral membrane protein LIMP‐II and that this binding depends on a DEXXXLI sequence in the LIMP-II tail, which points to a function of AP‐ 3 in intracellular sorting to lysoomes and melanosomes of a subset of cargo proteins via di‐leucine‐based sorting motifs.
Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice.
- P. Saftig, E. Hunziker, K. von Figura
- Biology, MedicineProceedings of the National Academy of Sciences…
- 10 November 1998
Assaying the resorptive activity of cathepsin-K-deficient osteoclasts in vitro revealed this function to be severely impaired, which supports the contention that cathepsypsin K is of major importance in bone remodeling.
Molecular basis of different forms of metachromatic leukodystrophy.
- A. Polten, A. Fluharty, C. Fluharty, J. Kappler, K. von Figura, V. Gieselmann
- MedicineThe New England journal of medicine
- 3 January 1991
Like many lysosomal storage disorders, metachromatic leukodystrophy shows clinical heterogeneity that seems to reflect genetic heterogeneity.
Crystal structure of human arylsulfatase A: the aldehyde function and the metal ion at the active site suggest a novel mechanism for sulfate ester hydrolysis.
The structural basis for understanding a novel mechanism of ester hydrolysis is provided and the functional importance of the unusually modified amino acid is explained.
Carbohydrate-deficient glycoprotein syndrome type Ib. Phosphomannose isomerase deficiency and mannose therapy.
Using standard diagnostic procedures, the disorder is indistinguishable from CDGS type Ia (phosphomannomutase deficiency).
Role of LAMP-2 in lysosome biogenesis and autophagy.
The data indicate that in hepatocytes LAMP-2 deficiency either directly or indirectly leads to impaired recycling of 46-kDa mannose 6-phosphate receptors and partial mistargeting of a subset of lysosomal enzymes.
Lysosomal enzymes and their receptors.
The Golgi is an ideal environment for the synthesis and modification of 6-Phosphate Residues and the role of these modifications in the development of LYSOSOMAL ENZYMES is under investigation.