Intestacy Reforms - The Way Things Were, 1952

Intestacy Reforms - The Way Things Were, 195

Privatizing the Family: The Reform of Child Law

Privatizing the Family: The Reform of Child La

The Functional Basis for Hemophagocytic Lymphohistiocytosis in a Patient with Co-inherited Missense Mutations in the Perforin (PFN1) Gene

About 30% of cases of the autosomal recessive immunodeficiency disorder hemophagocytic lymphohistiocytosis are believed to be caused by inactivating mutations of the perforin gene. We expressed perforin in rat basophil leukemia cells to define the basis of perforin dysfunction associated with two mutations, R225W and G429E, inherited by a compound heterozygote patient. Whereas RBL cells expressing wild-type perforin (67 kD) efficiently killed Jurkat target cells to which they were conjugated, the substitution to tryptophan at position 225 resulted in expression of a truncated (∼45 kD) form of the protein, complete loss of cytotoxicity, and failure to traffic to rat basophil leukemia secretory granules. By contrast, G429E perforin was correctly processed, stored, and released, but the rat basophil leukemia cells possessed reduced cytotoxicity. The defective function of G429E perforin mapped downstream of exocytosis and was due to its reduced ability to bind lipid membranes in a calcium-dependent manner. This study elucidates the cellular basis for perforin dysfunctions in hemophagocytic lymphohistiocytosis and provides the means for studying structure–function relationships for lymphocyte perforin

Cretney and Probert's family law [8th edition]

Cretney's Family Law covers both contemporary and historical principles of substantive family law, discusses the role of judicial discretion and critically evaluates the strengths and weaknesses of Family Law. All of the major elements are broken down into digestible sections that mirror closely the modular course structure and the learning requirements of students

Cretney's Family Law [5th edition]

The fifth edition combines exposition of themes and principles with an account of how the law works in practice. It provides up-to-date information, including the Adoption and Children Act 2002, and the proposed reforms relating to civil partnerships and the rights of transsexuals to marry