of <500 kb. ABP 280 is an attractive candidate gene 
for EDMD, as it is a structural protein with an amino 
portion quite similar to the dystrophin protein re- 
sponsible for DMD and BMD. Similar to dystrophin, 
much of the central protein is composed of a repeat- 
ing motif. Expression of the ABP 280 protein is ubiq- 
uitous among tissues, including muscle and heart, 
and various isoforms exist. Current efforts involve 
the identification of muscle-specific ABP 280 exons 
and the search for mutations in EDMD families. 
Xq28 Mapping 
Efforts continue to fine-map the terminal band of 
the human X chromosome Xq28, which lies imme- 
diately distal to the fragile X site. In the past year, an 
Xq28 physical map of 9 Mb was constructed with 
Drs. Annemarie Poustka (Heidelberg) and Hans 
Lehrach (Imperial Cancer Research Fund, London). 
Highly polymorphic loci (DXS455 and DXS548) 
were also cloned and mapped to this region, as was 
the human biglycans gene. Further refinement of 
the map positions of X-linked hydrocephalus and 
nephrogenic diabetes insipidus in Xq28 was accom- 
plished as well. (The projects in this section were 
supported by a grant from the National Institutes of 
Health.) 
Dr. Warren is also Associate Professor of Bio- 
chemistry and of Pediatrics at Emory University 
School of Medicine. 
Books and Chapters of Books 
Warren, S.T. 1991 . Molecular and somatic cell ge- 
netic approaches to the fragile X syndrome. In 
Molecular Genetic Approaches to Neuropsychi- 
atric Disease (Brosius, J., and Fremeau, R.T., 
Eds.). San Diego, CA: Academic, pp 349-366. 
Articles 
Consalez, G.G., Stayton, C.L., Freimer, N.B., Goone- 
wardena. P., Brown, W.T., Gilliam, T.C., and 
Warren, S.T. 1992. Isolation and characteriza- 
tion of a highly polymorphic human locus 
(DXS455) in proximal Xq28. Genomics 12: 
710-714. 
Fu, Y.-H., Kuhl, D.P.A., Pizzuti, A., Pieretti, M., Sut- 
cliffe, J.S., Richards, S., Verkerk, A.J.M.H., Hol- 
den, J.J.A., Fenwick, R.G., Jr., Warren, S.T., Oos- 
tra, B.A., Nelson, D.L., and Caskey, C.T. 1991. 
Variation of the CGG repeat at the fragile X site 
results in genetic instability: resolution of the 
Sherman paradox. Cell 67:1047-1058. 
Poustka, A., Dietrich, A., Langenstein, G., Toniolo, 
D., Warren, S.T., and Lehrach, H. 1991 . Physical 
map of human Xq27-qter: localizing the region of 
the fragile X mutation. Proc Natl Acad Sci USA 
88:8302-8306. 
Riggins, G.J., Sherman, S.L., Oostra, B.A., Sutcliffe, 
J.S., Feitell, D., Nelson, D.L., van Oost, B.A., 
Smits, A.P.T., Ramos, F.J., Pfender, E., Kuhl, D., 
Caskey, C.T., and Warren, S.T. 1992. Character- 
ization of a highly polymorphic dinucleotide re- 
peat 150 kb proximal to the fragile X site. Am f 
Med Genet 43:237-243. 
Sutclifife, J.S., Zhang, F., Nelson, D.L., Caskey, 
C. T., and Warren, S.T. 1992. PGR amplification 
and analysis of yeast artificial chromosomes. Ge- 
nomics 13:1303-1306. 
Traupe, H., van den Ouweland, A.M.W., van Oost, 
B.A., Vogel, W., Vetter, U., Warren, S.T., Rocchi, 
M., Darlison, M.G., and Ropers, H.H. 1992. Fine 
mapping of the human biglycan (BGN) gene 
within the Xq28 region employing a hybrid cell 
panel. Genomics 13:481-483. 
Van den Ouweland, A.M.W., Knoop, M.T., Knoers, 
V.V.A.M., Markslag, P.W.B., Rocchi, M., Warren, 
S.T., Mandel, J.L., Ropers, H.H., Fahrenholz, F., 
Monnens, L.A.H., and Van Oost, B.A. 1992. Colo- 
calization of the gene for nephrogenic diabetes 
insipidus and the vasopressin type-2 receptor 
gene in the Xq28 region. Genomics 13:1350- 
1352. 
Verkerk, A.J.M.H., deVries, B.B.A., Niermeijer, M.F., 
Nelson, D.L., Warren, S.T., Majoor-Krakauer, 
D. F., Halley, D.J.J. , and Oostra, B.A. 1992. Intra- 
genic probe used for diagnostics in fragile X fami- 
lies. Am J Med Genet 43:192-196. 
Willems, P.J., Vits, L., Raeymaekers, P., Beuten, J., 
Coucke, P., Holden, J.J.A., Van Broeckhoven, C, 
Warren, S.T., Sagi, M., Robinson, D., Dennis, N., 
Friedman, K.J., Magnay, D., Lyonnet, S., White, 
B.N., Wittwer, B.H., Aylsworth, A.S., and Reicke, 
S. 1992. Further localization of X-linked hydro- 
cephalus in the chromosomal region Xq28. Am f 
Hum Genet 51:501-515. 
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