Välimäki S, Forsberg L, Farnebo L-O, Larsson C
Department of Molecular Medicine, Endocrine Tumor Unit, CMM L8:01, Karolinska Hospital, SE-171 76 Stockholm, Sweden.
Int J Oncol. 2002 Oct;21(4):727-35.
Primary hyperparathyroidism is a common endocrine disease with a multifaceted genetic background, the elucidation of which has only begun. Among others, loss of the short arm of chromosome 1 and somatic inactivation of the multiple endocrine neoplasia type 1 gene (MEN1) in 11q13 represent significant alterations in the tumorigenesis. In the present study deletions of 1p were characterized and the findings were evaluated in relation to the loci of MEN1 and histone deacetylase 1 gene (HDAC1), a menin interacting partner in 1p, as well as to the clinical characteristics. Overall 1p LOH was detected in 18 of the 42 tumors analyzed (43%), and from the deletion patterns a main target interval of 40 cM was identified within 1p band 32.3-36.2. The mapping of HDAC1 centromeric of the main interval, and the lack of altered mRNA expression in tumors with LOH, suggest that HDAC1 is not the main target for 1p deletions in parathyroid tumors. Twenty-five of the 42 tumors (60%) showed alteration of either 1p, of the MEN1 locus, or both. Tumors with LOH at 11q13 had a significantly higher weight than tumors with 1p LOH. In conclusion, LOH in primary sporadic parathyroid adenomas occur frequently on the distal part of chromosome 1p and are thus clearly different from parathyroid carcinomas where the deletions are more proximally located. The findings support that the short arm of chromosome 1 harbors at least two different tumor suppressor genes involved in parathyroid tumorigenesis, the exact identification of which may provide a molecular basis for differential diagnosis of benign and malignant disease in the future.
原发性甲状旁腺功能亢进症是一种具有多方面遗传背景的常见内分泌疾病,对其的阐释才刚刚开始。其中,1号染色体短臂的缺失以及11q13区域多发性内分泌腺瘤1型基因(MEN1)的体细胞失活在肿瘤发生过程中代表了显著的改变。在本研究中,对1p缺失进行了特征描述,并结合MEN1和组蛋白去乙酰化酶1基因(HDAC1)(1p上与menin相互作用的伙伴)的基因座以及临床特征对研究结果进行了评估。在分析的42个肿瘤中,共检测到18个(43%)存在1p杂合性缺失(LOH),根据缺失模式在1p32.3 - 36.2带内确定了一个40 cM的主要目标区间。HDAC1定位于主要区间的着丝粒侧,且在发生LOH的肿瘤中mRNA表达未改变,这表明HDAC1不是甲状旁腺肿瘤中1p缺失的主要靶点。42个肿瘤中有25个(60%)显示1p、MEN1基因座或两者均发生改变。11q13发生LOH的肿瘤重量显著高于发生1p LOH的肿瘤。总之,原发性散发性甲状旁腺腺瘤中的LOH常见于1号染色体1p的远端,因此明显不同于甲状旁腺癌,后者的缺失位置更靠近近端。这些发现支持1号染色体短臂含有至少两个参与甲状旁腺肿瘤发生的不同肿瘤抑制基因,准确鉴定这些基因可能为未来良性和恶性疾病的鉴别诊断提供分子基础。