TY  - JOUR
A1  - Gessler, Manfred
A1  - Bruns, G. A. P.
T1  - A physical map around the WAGR complex on the short arm of chromosome 11
N2  - A long-range restriction map of part of the short arm of ehromosome 11 including the WAGR region has been constructed using pulsed-field gel electrophoresis and a number of infrequently cutting restriction enzymes. A total of 15.4 Mbp has been mapped in detall, extending from proximal 11p14 to the distal part of 11p12. The map localizes 35 different DNA probes and reveals at least nine areas with features eharaeteristle of BTF islands, some of which may be candidates for the different loci underlying the phenotype of the WAGR syndrome. This map will furthermore allow screening of DNA from individuals with WAGR-related phenotypes and from Wilms tumors for associated chromosomal rearrangements.
KW  - Biochemie
Y1  - 1989
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59246
ER  - 
TY  - JOUR
A1  - van Heyningen, V.
A1  - Bickmore, W. A.
A1  - Seawright, A.
A1  - Fletcher, J. M.
A1  - Maule, J.
A1  - Fekete, G.
A1  - Gessler, Manfred
A1  - Bruns, G. A.
A1  - Huerre-Jeanpierre, C.
A1  - Junien, C.
T1  - Role for the Wilms tumor gene in genital development?
N2  - No abstract available
KW  - Biochemie
Y1  - 1990
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59238
ER  - 
TY  - JOUR
A1  - Gessler, Manfred
A1  - Hameister, H.
A1  - Henry, I.
A1  - Junien, C.
A1  - Braun, T.
A1  - Arnold, H. H.
T1  - The human MyoD1 (MYF3) gene maps on the short arm of chromosome 11 but is not associated with the WAGR locus or the region for the Beckwith-Wiedemann syndrome
N2  - The human gene encoding the myogenic determination factor myf3 (mouse MyoD1) has been mapped to the short arm of chromosome 11. Analysis of several somatic cell hybrids containing various derivatives with deletions or translocations revealed that the human MyoD (MYF3) gene is not associated with the WAGR locus at chromosomal band 11pl3 nor with the loss of the heterozygosity region at 11p15.5 related to the Beckwith-Wiedemann syndrome. Subregional mapping by in situ hybridization with an myf3 specific probe shows that the gene resides at the chromosomal band llp14, possibly at llp14.3.
KW  - Biochemie
Y1  - 1990
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59221
ER  - 
TY  - JOUR
A1  - Vortkamp, A.
A1  - Thias, U.
A1  - Gessler, Manfred
A1  - Rosenkranz, W.
A1  - Kroisel, P. M.
A1  - Tommerup, N.
A1  - Kruger, G.
A1  - Gotz, J.
A1  - Pelz, L.
A1  - Grzeschik, Karl-Heinz
T1  - A somatic cell hybrid panel and DNA probes for physical mapping of human chromosome 7p
N2  - No abstract available
KW  - Biochemie
Y1  - 1991
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59217
ER  - 
TY  - JOUR
A1  - Wolf, Markus
A1  - Klug, Jörg
A1  - Hackenberg, Reinhard
A1  - Gessler, Manfred
A1  - Grzeschik, Karl-Heinz
A1  - Beato, Miguel
A1  - Suske, Guntram
T1  - Human CC10, the homologue of rabbit uteroglobin: genomic cloning, chromosomal localization and expression in endometrial cell lines
N2  - No abstract available
KW  - Biochemie
Y1  - 1992
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59206
ER  - 
TY  - JOUR
A1  - Gessler, Manfred
A1  - König, A.
A1  - Bruns, G. A. P.
T1  - The genomic organization and expression of the WT1 gene
N2  - The Wilms tumor gene WTl, a proposed tumor suppressor gene, has been identifled based on its location within a homozygous deletion found in tumor tissue. The gene encodes a putative transcription factor containing a Cys/His zinc finger domain. The critical homozygous deletions, however, are rarely seen, suggesting that in many cases the gene may be inactivated by more subtle alterations. To facilitate the seareh for smaller deletions and point mutations we have established the genomic organization of the WTl gene and have determined the sequence of all 10 exons and flanking intron DNA. The pattern of alternative splicing in two regions has been characterized in detail. These results will form the basis for future studies of mutant alleles at this locus.
KW  - Biochemie
Y1  - 1992
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59195
ER  - 
TY  - JOUR
A1  - Gessler, Manfred
A1  - Grupe, Andrew
A1  - Grzeschik, Karl-Heinz
A1  - Pongs, Olaf
T1  - The potassium channel gene HK1 maps to human chromosome 11p14.1, close to the FSHB gene
N2  - Transiently activating (A-type) potassium (K) channels are important regulators of action potential and action potential firing frequencies. HK1 designates the firsthuman cDNA that is highly homologous to the rat RCK4 cDNA that codes for an A-type K-channel. The HK1 channel is expressed in heart. By somatic cell hybrid analysis, the HK1 gene has been assigned to human chromosome 11p13-pl4, the WAGR deletion region (Wilms tumor, aniridia, genito-urinary abnormalities and mental retardation). Subsequent pulsed field gel (PFG) analysis and comparison with the well-established PFG map of this region localized the gene to 11p14, 200-600 kb telomeric to the FSHB gene.
KW  - Biochemie
Y1  - 1992
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59184
ER  - 
TY  - JOUR
A1  - Poulat, F.
A1  - Morin, D.
A1  - Konig, A.
A1  - Brun, P.
A1  - Giltay, J.
A1  - Sultan, C.
A1  - Dumas, R.
A1  - Gessler, Manfred
A1  - Berta, P.
T1  - Distinct molecular origins for Denys-Drash and Frasier syndromes
N2  - The direct involvment of the Wilm's tumor suppressor gene (WTl) in Denys-Drash syndrome through mutations within exons 8 or 9 has recently been established. The absence of such alterations in three patients with Frasier syndrome provides a molecular basis for distinguishing these two syndromes that are associated with streak gonads, pseudohermaphroditism and renal failure.
KW  - Biochemie
Y1  - 1993
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59172
ER  - 
TY  - JOUR
A1  - Konig, Anja
A1  - Jakubiczka, Sybille
A1  - Wieacker, Peter
A1  - Schlösser, Hans W.
A1  - Gessler, Manfred
T1  - Further evidence that imbalance of WT1 isoforms may be involved in Denys-Drash syndrome
N2  - No abstract available
KW  - Biochemie
Y1  - 1993
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59167
ER  - 
TY  - JOUR
A1  - Henry, Isabelle
A1  - Hoovers, Jan
A1  - Barichard, Fernande
A1  - Berthéas, Marie-Francoise
A1  - Puech, Anne
A1  - Prieur, Fabienne
A1  - Gessler, Manfred
A1  - Bruns, Gail
A1  - Mannens, Marcel
A1  - Junien, Claudine
T1  - Pericentric intrachromosomal insertion responsible for recurrence of del(11)(p13p14) in a family
N2  - The combined use of qualitative and quantitative analysis of I I p I 3 polymorphic markers tagether with chromosomal in situ suppression hybridization (CISS) with biotin labeled probes mapping to I I p allowed us to characterize a complex rearrangement segregating in a family. We detected a pericentric intrachromosomal insertion responsible (or recurrence of del( I I )(p 13p 14) in the family: an insertion of band I I p 13-p 14 carrying the genes for predisposition to Wilms' tumor, WT I, and for aniridia, AN2, into the long arm of chromosome I I in II q 13-q 1<4. Asymptomatic balanced carriers were observed over three generations. Classical cytogenetics had failed to detect this anomaly in the balanced carriers, who were first considered to be somatic mosaics for del( II )(p 13). Two of these women gave birth to children carrying a deleted chromosome II. most likely resulting from the loss of the I I p 13 band inserted in I I q. Although in both cases the deletion encompassed exactly the same maternally inherited markers, there was a wide Variation in clinical expression. One child, with the karyotype 46,XY,del(ll)(pllpl4), presented the full-blown WAGR syndrome with anlridia, mental retardation, Wilms' tumor, and pseudohermaphroditism, but also had proteinuria and glomerular sclerosis reminiscent of Drash syndrome. In contrast, the other one, a girl with the karyotype 46,XX,del( I I )(p I 3), only had aniridia. Although a specific set of mutational sites has been observed in Drash patients, these findings suggest that the loss of one copy of the WTI gene can result in similar genital and kidney abnormalities.
KW  - Biochemie
Y1  - 1993
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-59157
ER  -