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22 results on '"Ferrari, David M."'

4. Geomorphology and Plate Tectonics

Author

Guiseppi, Antonio R., Ferrari, David M., Guiseppi, Antonio R., and Ferrari, David M.

Subjects
Plate tectonics, Landforms, Geomorphology
Abstract

Geomorphology is the scientific study of landforms and the processes that shape them whereas plate tectonics specifically describes the large scale motions of Earth's lithosphere. This new book presents theory, methodology and applications, as well as studies of specific geographic regions. A model to evaluate the effect of groundwater fluctuations on the development of gullies and hence, on landscape evolution is analyzed. The use of cosmogenic nuclides in geomorphology and earth-surface processes is also reviewed and uncertainties and limitations related to current understanding of the physical properties of cosmogenic nuclides are summarized. In addition, this book studies plate tectonics in several regions of the world, including Siberia, Western North America, North Africa, India, and Northwest Australia.

Published
2009

19. Identification of a Novel Saturable Endoplasmic Reticulum Localization Mechanism Mediated by the C-Terminus of aDictyosteliumProtein Disulfide Isomerase

Author

Monnat, Jean, Neuhaus, Eva M., Pop, Marius S., Ferrari, David M., Kramer, Barbara, and Soldati, Thierry

Abstract

Localization of soluble endoplasmic reticulum (ER) resident proteins is likely achieved by the complementary action of retrieval and retention mechanisms. Whereas the machinery involving the H/KDEL and related retrieval signals in targeting escapees back to the ER is well characterized, other mechanisms including retention are still poorly understood. We have identified a protein disulfide isomerase (Dd-PDI) lacking the HDEL retrieval signal normally found at the C terminus of ER residents in Dictyostelium discoideum. Here we demonstrate that its 57 residue C-terminal domain is necessary for intracellular retention of Dd-PDI and sufficient to localize a green fluorescent protein (GFP) chimera to the ER, especially to the nuclear envelope. Dd-PDI and GFP-PDI57 are recovered in similar cation-dependent complexes. The overexpression of GFP-PDI57 leads to disruption of endogenous PDI complexes and induces the secretion of PDI, whereas overexpression of a GFP-HDEL chimera induces the secretion of endogenous calreticulin, revealing the presence of two independent and saturable mechanisms. Finally, low-level expression of Dd-PDI but not of PDI truncated of its 57 C-terminal residues complements the otherwise lethal yeast TRG1/PDI1 null mutation, demonstrating functional disulfide isomerase activity and ER localization. Altogether, these results indicate that the PDI57 peptide contains ER localization determinants recognized by a conserved machinery present in D. discoideumand Saccharomyces cerevisiae.

Published
2000
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20. Crystal Structure and Functional Analysis of Drosophila Wind, a Protein-disulfide Isomerase-related Protein.

Author

Qingjun Ma, Chaoshe Guo, Lars C., Barnewitz, Kathrin, Sheldrick, George M., Söling, Hans-Dieter, Usón, Isabel, and Ferrari, David M.

Subjects
*PROTEINS, *ISOMERASES, *DROSOPHILA melanogaster
Abstract

In the developing Drosophila melanogaster embryo, dorsal-ventral patterning displays an absolute requirement for the product of the essential windbeutel gene, Wind. In homozygous windbeutel mutant flies, dorsalventral patterning fails to initiate because of the failure of the Golgi-resident proteoglycan-modifying protein, Pipe, to exit the endoplasmic reticulum, and this leads to the death of the embryo. Here, we describe the threedimensional structure of Wind at 1.9-Å resolution and identify a candidate surface for interaction with Pipe. This represents the first crystal structure of a eukaryotic protein-disulfide isomerase-related protein of the endoplasmic reticulum to be described. The dimeric protein is composed of an N-terminal thioredoxin domain and a C-terminal α-helical domain unique to protein-disulfide isomerase D proteins. Although Wind carries a CXXC motif that is partially surface accessible, this motif is redox inactive, and the cysteines are not required for the targeting of Pipe to the Golgi. However, both domains are required for targeting Pipe to the Golgi, and, although the mouse homologue ERp28 cannot replace the function of Wind, exchange of the Wind D-domain with that of ERp28 allows for efficient Golgi transport of Pipe. [ABSTRACT FROM AUTHOR]

Published
2003
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21. Hereditary angioedema in a single family with specific mutations in both plasminogen and SERPING1 genes.

Author

Bork K, Zibat A, Ferrari DM, Wollnik B, Schön MP, Wulff K, and Lippert U

Subjects
Adolescent, Adult, Child, Child, Preschool, Family, Female, Humans, Male, Middle Aged, Mutation, Missense, Phenotype, Young Adult, Angioedemas, Hereditary genetics, Complement C1 Inhibitor Protein genetics, Mutation, Plasminogen genetics
Abstract

Background: Hereditary angioedema (HAE) is a group of genetic diseases characterized by recurrent, painful and potentially lethal tissue swelling. The most common form results from mutations in the SERPING1 gene, leading to reduced function of complement 1 inhibitor (C1-INH). Rarer forms with normal C1-INH may arise from mutations in the coagulation factor F12 gene, but mostly the genetic background is unknown. Recently, a novel HAE mutation in the plasminogen (PLG) gene was shown., Patients and Methods: We analyzed the various clinical manifestations of HAE in 14 related patients using clinical data, biochemical analysis for C1-INH and C4 as well as gene sequencing., Results: Patients' symptoms were assigned to two different forms of HAE. In ten patients suffering from swelling of the lips or tongue but not of the extremities, a mutation in the PLG gene (c.988A>G) was found whereas in the only four patients with swelling of the gastrointestinal tract and extremities, a mutation in the SERPING1 gene (c.1480C>T) was identified. In two cases this was additional to PLG c.988A>G., Conclusions: This unique finding of two different HAE-specific mutations in a large family not only explains the divergent phenotypes but also supports a genotype-phenotype correlation showing that abdominal attacks and swelling of the extremities are common with HAE-C1-INH but unusual with HAE-PLG., (© 2020 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Published
2020
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