04160nam 2200685 a 450 99620696720331620230227172147.01-280-78379-697866136941880-470-72051-40-470-71819-6(CKB)1000000000688119(EBL)703890(OCoLC)797918439(SSID)ssj0000715456(PQKBManifestationID)11472760(PQKBTitleCode)TC0000715456(PQKBWorkID)10720160(PQKB)10282960(MiAaPQ)EBC703890(Au-PeEL)EBL703890(CaPaEBR)ebr10575491(CaONFJC)MIL369418(EXLCZ)99100000000068811920790621d1979 uy 0engur|n|---|||||txtrdacontentcrdamediacrrdacarrierEnzyme defects and immune dysfunctionAmsterdam ;New York Excerpta Medica19791 online resource (301 pages) illustrationsCiba Foundation symposium. New series ;68"Symposium on Enzyme Defects and Immune Dysfunction, held at the Ciba Foundation, London, 7th-9th November 1978"--Participants page.0-470-66379-0 0-444-90088-8 Includes bibliographical references and indexes.Enzyme Defects and Immune Dysfunction; Contents; Chairman's introduction; Adenosine deaminase and purine nucleoside phosphorylase deficiency: how they were discovered and what they may mean; Discussion; Buridan's ass: one man's view of the immune system; Discussion; Clinical delineation of adenosine deaminase deficiency; Discussion; Immunological aberrations in purine nucleoside phosphorylase deficiencies; Discussion; Immunodeficiency due to transcobalamin II deficiency; Discussion; General Discussion I Model systems for study of enzyme deficienciesNucleotide metabolism in cultured T cells and in cells of patients deficient in adenosine deaminase and purine nucleoside phosphorylase Discussion; Deoxyribonucleoside toxicity in adenosine deaminase and purine nucleoside phosphorylase deficiency: implications for the development of new immunosuppressive agents; Discussion; Ecto 5'-nucleotidase deficiency in primary hypogammaglobulinaemia; Discussion; General Discussion II The methylation hypothesis of adenosine toxicity; Enzymology of immunodeficiency states; Allosteric regulation of calf thymus ribonucleotide reductase; DiscussionAdenosine deaminase: characteristics of the normal and mutant forms of the human enzyme Discussion; Purine nucleoside phosphorylase: the normal enzyme and structural alterations in immunodeficiency disease; Discussion; Purine nucleoside phosphorylase : immunodetection and characterization of the human enzyme; Enzyme replacement and other biochemical approaches to the therapy of adenosine deaminase deficiency; Discussion; An approach to the restoration of T cell function in a purine nucleoside phosphorylase deficient patient; Discussion; Purine metabolism in adenosine deaminase deficiencyFinal General Discussion Selective treatment of lymphoid malignancy with adenosine deaminase inhibitors Defects in differentiation underlying ADA and PNP deficiency; Search for new enzyme deficiencies; Index of contributors; Subject indexCiba Foundation symposium.New series ;68.Immunological deficiency syndromesCongressesAdenosine deaminaseCongressesPurinesMetabolismCongressesEnzymesCongressesImmunological deficiency syndromesAdenosine deaminasePurinesMetabolismEnzymes616.3/99Symposium on Enzyme Defects and Immune Dysfunction(1978 :London, England)MiAaPQMiAaPQMiAaPQBOOK996206967203316Enzyme defects and immune dysfunction2145091UNISA01599nam a2200313 i 4500991000752589707536100531s2008 gw b 100 0 eng d9783832939618 (pbk.)383293961X (pbk.)b13906057-39ule_instDip.to Studi Giuridiciitaengfreger944.04In the embrace of France :the law of nations and constitutional law in the French satellite states of the revolutionary and Napoleonic age, (1789-1815) : acts of the international conference held at Tilburg University on 27 & 28 April 2006 /Beatrix Jacobs, Raymond Kubben, Randall Lesaffer, edsBaden-Baden :Nomos,2008175 p. ;23 cm.Studien zur Geschichte des Vèolkerrechts ;Bd. 18Include riferimenti bibliograficiDiritto internazionaleStoriaSec. 18.CongressiDiritto internazionaleStoriaSec. 19.CongressiJacobs, B. C. M.(Beatrix C. M.)authorhttp://id.loc.gov/vocabulary/relators/aut784964Kubben, Raymondauthorhttp://id.loc.gov/vocabulary/relators/aut731416Lesaffer, Randallauthorhttp://id.loc.gov/vocabulary/relators/aut509696.b1390605702-04-1431-05-10991000752589707536LE027 944.04 JAC01.01 12027000242221le027-E54.00-l- 00000.i1519089428-10-10In the embrace of France1747374UNISALENTOle02731-05-10ma -enggw 00