September 1997

volume 17 Number 1     editorial   Holes in the Swiss legislation?  p 1   news & views   Save the males!  p 3 David C. Page   From mighty mice to mighty cows   p 4 Mark Westhusin   A tenascin knockout with a phenotype   p 5 Harold P. Erickson   Mom1 leads the pack   p 7 Tommaso A. Dragani & Giacomo Manenti   correspondence   Heterozygote advantage for HLA class-II type in hepatitis B virus infection  p 10 Mark R.Thursz1, Howard C.Thomas1, Brian M. Greenwood2 & Adrian V.S. Hill3   Imprinting of the Angelman syndrome gene, UBE3A, is restricted to brain  p 12 Thanh H. Vu & Andrew R. Hoffman   The Angelman syndrome candidate gene, UBE3A/E6-AP, is imprinted in brain  p 14 Claire Rougeulle1?3, Heather Glatt1 & Marc Lalande1?3   Stargardt's ABCR is localized to the disc membrane of retinal rod outer segments  p 15 Hui Sun1 & Jeremy Nathans1,2   What is expanded in progressive myoclonus epilepsy?  p 17 Maria D. Lalioti1, Hamish S. Scott1, & Stylianos E. Antonarakis1,2   Mutations in CDMP1 cause autosomal dominant brachydactyly type C  p 18 Alexander Polinkovsky1, Nathaniel H. Robin1?3, J. Terrig Thomas4, Mira Irons5, Audrey Lynn1, Frances R. Goodman6, William Reardon7, Sarina G. Kant8, Han G. Brunner9, Ineke van der Burgt9, David Chitayat10, Julie McGaughran11, Dian Donnai11, Frank P. Luyten4, & Matthew L. Warman1?3   Ethics and genetics in China: an inside story  p 20 Xin Mao   new technology   The use of a genetic map of biallelic markers in linkage studies  p 21 Leonid Kruglyak   articles   A candidate gene for familial Mediterranean fever  p 25 The French FMF Consortium   DMBT1, a new member of the SRCR superfamily, on chromosome 10q25.3­26.1 is deleted in malignant brain tumours   p 32 Jan Mollenhauer1, Stefan Wiemann1, Wolfram Scheurlen2, Bernhard Korn1, Yutaka Hayashi3, Klaus K. Wilgenbus1*, Andreas von Deimling3 & Annemarie Poustka1   The early-onset torsion dystonia gene (DYT1) encodes an ATP-binding protein   p 40 Laurie J. Ozelius1, Jeffrey W. Hewett1, Curtis E. Page1, Susan B. Bressman2, Patricia L. Kramer3, Christo Shalish1, Deborah de Leon2, Mitchell F. Brin5, Deborah Raymond2, David P. Corey4, Stanley Fahn2, Neil J. Risch6, Alan J. Buckler1, James F. Gusella1 & Xandra O. Breakefield1   A mouse model for Zellweger syndrome  p 49 Myriam Baes1, Pierre Gressens2, Eveline Baumgart3, Peter Carmeliet4, Minne Casteels5, Marc Fransen5, Philippe Evrard2, Dariush Fahimi3, Peter E. Declercq1, Désiré Collen4, Paul P. van Veldhoven5 & Guy P. Mannaerts5   Disruption of human limb morphogenesis by a dominant negative mutation in CDMP1   p 58 J. Terrig Thomas1, Michael W. Kilpatrick2, Keming Lin1, Ludwig Erlacher1, Peter Lembessis2, Teresa Costa3, Petros Tsipouras2 & Frank P. Luyten1   Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion  p 65 Gilles David1, Nacer Abbas1, Giovanni Stevanin1, Alexandra Dürr1,2, Gaël Yvert3, Géraldine Cancel1, Chantal Weber3, Georges Imbert3, Frédéric Saudou3, Eric Antoniou4, Harry Drabkin4, Robert Gemmill4, Paola Giunti5, Ali Benomar6, Nick Wood5, Merle Ruberg1, Yves Agid1,2, Jean-Louis Mandel3 & Alexis Brice1,2   letters   A deletion in the bovine myostatin gene causes the double-muscled phenotype in cattle  p 71 Luc Grobet1, Luis José Royo Martin2, Dominique Poncelet1, Dimitri Pirottin1, Benoit Brouwers1, Juliette Riquet1, Andreina Schoeberlein1, Susana Dunner2, François Ménissier3, Julio Massabanda4, Ruedi Fries4, Roger Hanset1 & Michel Georges1   Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons   p 75 Urs Albrecht1, James S. Sutcliffe2, Bruce M. Cattanach5, Colin V. Beechey5, Dawna Armstrong3, Gregor Eichele1 & Arthur L. Beaudet2,4   Familial colorectal cancer in Ashkenazim due to a hypermutable tract in APC   p 79 Steven J. Laken1, Gloria M. Petersen1,2, Stephen B. Gruber1, Carole Oddoux3, Harry Ostrer3, Francis M.Giardiello4, Stanley R. Hamilton1,5, Heather Hampel6, Arnold Markowitz7, David Klimstra8, Suresh Jhanwar9, Sidney Winawer7, Kenneth Offit6, Michael C. Luce10, Kenneth W. Kinzler1 & Bert Vogelstein1,11   Mapping of a familial essential tremor gene, FET1, to chromosome 3q13  p 84 Jeffrey R. Gulcher1, Io orlákur Jónsson1, Augustine Kong1,2, Kristleifur Kristjánsson1, Michael L. Frigge1,2, Ari Kárason1, Ingibjörg E. Einarsdóttir1, Hreinn Stefánsson1, Anna S. Einarsdóttir1, Sigrún Sigurgardóttir1, Sigurgur Baldursson1, Sóley Björnsdóttir1, Soffía M. Hrafnkelsdóttir1, Finnbogi Jakobsson3, John Benedickz1,4 & Kári Stefánsson1   Secretory phospholipase Pla2g2a confers resistance to intestinal tumorigenesis  p 88 Robert T. Cormier1, Karen H. Hong2,3, Richard B. Halberg1, Trevor L. Hawkins2, Paul Richardson2, Rita Mulherkar4, William F. Dove1 & Eric S. Lander2,3   Epilepsy and mental retardation limited to females: an X-linked dominant disorder with male sparing   p 92 Stephen G. Ryan1,2, Phillip F. Chance1,2, Chang-Hua Zou1,2, Nancy B. Spinner2, Jeffrey A. Golden3 & Susan Smietana4   Clustering of missense mutations in the ataxia-telangiectasia gene in a sporadic T-cell leukaemia   p 96 Igor Vorechovsk ´y1,2, Liping Luo1, Martin J.S. Dyer3, Daniel Catovsky3, Peter L. Amlot2, John C. Yaxley2, Letizia Foroni2, Lennart Hammarström1, A. David B. Webster2 & Martin A.R. Yuille3   The Werner syndrome protein is a DNA helicase  p 100 Matthew D. Gray, Jiang-Cheng Shen, Ashwini S. Kamath-Loeb, A. Blank, Bryce L. Sopher, George M. Martin, Junko Oshima & Lawrence A. Loeb   Tenascin-X deficiency is associated with Ehlers-Danlos syndrome  p 104 Grant H. Burch1, Yan Gong1, Wenhui Liu1, Robert W. Dettman1, Cynthia J. Curry3, Lynne Smith4, Walter L. Miller1,2 & James Bristow1   Fusion of retinoic acid receptor alpha to NuMA, the nuclear mitotic apparatus protein, by a variant translocation in acute promyelocytic leukaemia   p 109 Richard A. Wells1,2*, Charles Catzavelos3 & Suzanne Kamel-Reid1,2   Female embryonic lethality in mice nullizygous for both Msh2 and p53  p 114 Aaron Cranston1, Tina Bocker1, Armin Reitmair2,4, Juan Palazzo3, Teresa Wilson1, Tak Mak2 & Richard Fishel1   Reverse genetics by chemical mutagenesis in Caenorhabditis elegans  p 119 Gert Jansen, Esther Hazendonk, Karen L. Thijssen & Ronald H.A. Plasterk