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201.
L J Valentijn F Baas R A Wolterman J E Hoogendijk N H van den Bosch I Zorn A W Gabre?ls-Festen M de Visser P A Bolhuis 《Nature genetics》1992,2(4):288-291
We have investigated the peripheral myelin protein gene, PMP-22, in a family with Charcot-Marie-Tooth disease type 1A (CMT1A). The DNA duplication commonly found in CMT1A was absent in this family, but strong linkage existed between the disease and the CMT1A marker VAW409R3 on chromosome 17p11.2. We found a point mutation in PMP-22 which was completely linked with the disease. The mutation, a proline for leucine substitution in the first putative transmembrane domain, is identical to that recently found in the Trembler-J mouse. The presence of this PMP-22 defect in this CMT1A family and the location of PMP-22 within the DNA duplication associated with CMT1A suggest that both structural alteration and overexpression of PMP-22 may lead to the disease. 相似文献
202.
J. K. Buitelaar H. van Engeland C. H. de Kogel H. de Vries J. A. R. A. M. van Hooff J. M. van Ree 《Cellular and molecular life sciences : CMLS》1992,48(4):391-394
When charting the structure of the social behavior of autistic children by means of an ethologically analyzed playroom session, deficits appeared in the reciprocity of eye-contact and in the location of verbal initiatives. These deficits in social behavior were beneficially influenced by treatment with the adrenocorticotrophic hormone (4–9) analog ORG 2766. 相似文献
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Germline mosaicism and Duchenne muscular dystrophy mutations 总被引:12,自引:0,他引:12
E Bakker C Van Broeckhoven E J Bonten M J van de Vooren H Veenema W Van Hul G J Van Ommen A Vandenberghe P L Pearson 《Nature》1987,329(6139):554-556
Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular disease with an incidence of approximately 1 in 3,500 newborn boys. The DMD locus has a high mutation frequency: one third of the cases is thought to result from a new mutation. Linkage studies using probes to detect restriction fragment length polymorphisms and DNA deletion studies have greatly improved DMD carrier detection and prenatal diagnosis. Here we report on two families in which a pERT87 (DXS164) deletion was transmitted to more than one offspring by women who showed no evidence for the mutation in their own somatic (white blood) cells. We also show that the deletion in both siblings in one of the families is identical, indicating that the deletion must have occurred during mitosis in early germline proliferation, leading to a germline mosaicism. This phenomenon may turn out to be a major factor contributing to the induction of DMD mutations, and has important implications for the counselling of DMD families. 相似文献
205.
J de Koning A M Tijssen J A van Dieten T R Koiter G A Schuiling G P van Rees 《Experientia》1986,42(1):60-62
The patterns of LH secretion during constant stimulation of the pituitary glands of estradiol-treated ovariectomized rats with a maximally stimulating amount of LH-RH in vivo and in vitro correspond with each other qualitatively and quantitatively. In vitro the changes with time of the LH secretion rate are somewhat retarded, especially the occurrence of desensitization. 相似文献
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207.
Deletion mapping of the inducible promoter of human IFN-beta gene 总被引:11,自引:0,他引:11
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