Fu S M
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 1989 Jun;11(3):165-9.
A 31 year-old male with Crouzon syndrome and a 21p+ was observed. Pedigree analysis and molecular cytogenetic study on the members of the family revealed that this syndrome was an autosomal dominant disease inherited from the proband's mother, and the 21p+ was transmitted from the father. Neither large AG-band nor double NOR was observed on p+. Using a 7.3 kb fragment of 18S and 28S rRNA genes as probe, chromosomal in situ hybridization demonstrated that this p+ was very probably the result of enlargement of NOR or duplication of rRNA genes on this chromosome.
观察到一名患有克鲁宗综合征和21号染色体短臂三体(21p+)的31岁男性。对该家族成员进行的系谱分析和分子细胞遗传学研究表明,这种综合征是一种常染色体显性疾病,从先证者的母亲遗传而来,而21p+则是从父亲遗传而来。在21号染色体短臂上未观察到较大的AG带或双随体。使用18S和28S rRNA基因的7.3 kb片段作为探针,染色体原位杂交表明,这个21号染色体短臂三体很可能是该染色体上核仁组织区(NOR)扩大或rRNA基因重复的结果。