neopentyloxythiocarbonylmercapto-acetic acid | 25646-04-2

• 分子结构分类: 有机化合物 - 脂质和类脂质分子 - 脂肪酰基
• 英文名称: neopentyloxythiocarbonylmercapto-acetic acid
• 英文别名: Neopentyloxythiocarbonylmercapto-essigsaeure；Neopentylxanthogenessigsaeure；<(Neopentyloxythiocarbonyl)-thio>-acetic acid
• CAS: 25646-04-2
• 化学式: C₈H₁₄O₃S₂
• 分子量: 222.329
• InChiKey: YIRJIWDVABDPJT-UHFFFAOYSA-N

计算性质

• 辛醇/水分配系数(LogP)：
  2.15
• 重原子数：
  13.0
• 可旋转键数：
  3.0
• 环数：
  0.0
• sp3杂化的碳原子比例：
  0.75
• 拓扑面积：
  46.53
• 氢给体数：
  1.0
• 氢受体数：
  4.0

反应信息

• 作为反应物：
  描述：

  neopentyloxythiocarbonylmercapto-acetic acid 在 sodium hydroxide 、 肼 作用下， 生成 Neopentyloxythiocarbonylhydrazin
  参考文献：
  名称：

  Jensen,K.A. et al., Acta Chemica Scandinavica (1947), 1969, vol. 23, p. 1916 - 1934

  摘要：

  DOI：
• 作为产物：
  描述：

  alkaline earth salt of/the/ methylsulfuric acid 、 alkaline earth salt of/the/ methylsulfuric acid 在 水 作用下， 生成 neopentyloxythiocarbonylmercapto-acetic acid
  参考文献：
  名称：

  Johansson, Arkiv foer Kemi, 1946, vol. 22 B, # 2, p. 4

  摘要：

  DOI：

文献信息

• Aggressive Metastatic Follicular Thyroid Carcinoma with Anaplastic Transformation Arising from a Long-Standing Goiter in a Patient with Pendred's Syndrome
  作者：Rosalinda Camargo、Eduardo Limbert、Mary Gillam、Maria Manuela Henriques、Carlos Fernandes、Ana Luisa Catarino、Jorge Soares、Venancio A.F. Alves、Peter Kopp、Geraldo Medeiros-Neto

  DOI：10.1089/105072501753211073

  日期：2001.10

  In this article we describe detailed pathological and molecular genetics studies in a consanguineous kindred with Pendred's syndrome. The index patient was a 53-year-old female patient with congenital deafness and goiter. Her parents were first-degree cousins. She had a large goiter (150 g) that had been present since childhood. One of her sisters and a niece are also deaf and have goiter as well. The presence of Pendred's syndrome was confirmed by a positive perchlorate test and the demonstration of a Mondini malformation. Thyroid function tests (under levothyroxine [LT4] therapy) were in the euthyroid range with a thyrotropin [TSH] level of 2.8 muU/mL (0.2-3.2), a serum total thyroxine (T-4) of 90 nmol/L (54-142), and a serum total triiodothyronine (T-3) of 2.7 nmol/L (0.8-2.4). Total thyroidectomy was performed, and the mass in the right lobe was found to have invaded adjacent tissues. The histopathological findings were consistent with a follicular carcinoma with areas of anaplastic transformation and lung metastasis. The patient was treated twice with 100 mCi (131)iodine (3,700 MBq) and received suppressive doses of LT4. Postoperatively, the serum thyroglobulin (Tg) levels remained markedly elevated (2,352 to 41,336 ng/mL). The patient died of a sudden severe episode of hemoptysis. Sequence analysis of the PDS gene performed with DNA from the two relatives with Pendred's syndrome revealed the presence of a deletion of thymidine 279 in exon 3, a point mutation that results in a frameshift and a premature stop codon at codon 96 in the pendrin molecule. We concluded that prolonged TSH stimulation because of iodine deficiency or dyshormonogenesis in combination with mutations of oncogenes and/or tumor suppressor genes, may result in the development of follicular thyroid carcinomas that undergo transformation into anaplastic cancers. It is likely that these pathogenetic mechanisms have been involved in the development of aggressive metastatic thyroid cancer in this unusual patient with Pendred's syndrome.