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来自一名患有拉布森 - 门登霍尔综合征患者的培养细胞中胰岛素结合能力下降:培养淋巴细胞与培养成纤维细胞研究结果的差异

Decreased insulin binding to cultured cells from a patient with the Rabson-Mendenhall syndrome: dichotomy between studies with cultured lymphocytes and cultured fibroblasts.

作者信息

Taylor S I, Underhill L H, Hedo J A, Roth J, Rios M S, Blizzard R M

出版信息

J Clin Endocrinol Metab. 1983 Apr;56(4):856-61. doi: 10.1210/jcem-56-4-856.

DOI:10.1210/jcem-56-4-856
PMID:6339538
Abstract

[125I]Insulin binding has been studied with cultured cells from an insulin-resistant patient with the Rabson-Mendenhall syndrome. Previously, this patient has been demonstrated to have decreased insulin binding to circulating blood cells. Similarly, [125I]insulin binding to cultured lymphocytes and cultured fibroblasts was markedly decreased. Whereas the decrease in [125I]insulin binding to cultured lymphocytes appeared to result from a decrease in receptor number in cultured lymphocytes, there appeared to be a reduction in the affinity of [125I]insulin binding to cultured fibroblasts. This discrepancy between the observations with the two cell types is a general phenomenon which has been described in patients with a variety of syndromes of extreme insulin resistance. It is possible that the interpretation of the studies with the fibroblasts is complicated by the confounding influence of receptors for insulin-like growth factors on the surface of those cells. If the number of insulin receptors is sufficiently reduced on fibroblasts from the patient with extreme insulin resistance, then the low level of residual [125I]insulin binding may involve primarily receptors for insulin-like growth factors, which bind insulin with relatively low affinity.

摘要

利用来自一名患有拉布森 - 门登霍尔综合征的胰岛素抵抗患者的培养细胞,对[125I]胰岛素结合进行了研究。此前已证实,该患者与循环血细胞的胰岛素结合减少。同样,[125I]胰岛素与培养的淋巴细胞和培养的成纤维细胞的结合也显著减少。虽然[125I]胰岛素与培养淋巴细胞的结合减少似乎是由于培养淋巴细胞中受体数量减少所致,但[125I]胰岛素与培养成纤维细胞的结合亲和力似乎有所降低。这两种细胞类型观察结果之间的差异是一种普遍现象,在患有各种极端胰岛素抵抗综合征患者中均有描述。成纤维细胞表面胰岛素样生长因子受体的混杂影响可能使对成纤维细胞研究的解释变得复杂。如果来自极端胰岛素抵抗患者的成纤维细胞上胰岛素受体数量充分减少,那么残余的低水平[125I]胰岛素结合可能主要涉及胰岛素样生长因子受体,该受体与胰岛素的结合亲和力相对较低。

相似文献

1
Decreased insulin binding to cultured cells from a patient with the Rabson-Mendenhall syndrome: dichotomy between studies with cultured lymphocytes and cultured fibroblasts.来自一名患有拉布森 - 门登霍尔综合征患者的培养细胞中胰岛素结合能力下降:培养淋巴细胞与培养成纤维细胞研究结果的差异
J Clin Endocrinol Metab. 1983 Apr;56(4):856-61. doi: 10.1210/jcem-56-4-856.
2
Insulin receptor degradation is accelerated in cultured lymphocytes from patients with genetic syndromes of extreme insulin resistance.在患有极端胰岛素抵抗遗传综合征患者的培养淋巴细胞中,胰岛素受体降解加速。
J Clin Invest. 1984 Oct;74(4):1366-74. doi: 10.1172/JCI111547.
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Insulin-receptor biosynthesis in cultured lymphocytes from an insulin-resistant patient (Rabson-Mendenhall syndrome). Evidence for defect before insertion of receptor into plasma membrane.胰岛素抵抗患者(拉布森-门登霍尔综合征)培养淋巴细胞中的胰岛素受体生物合成。受体插入质膜之前存在缺陷的证据。
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Decreased insulin binding in cultured lymphocytes from two patients with extreme insulin resistance.两名极端胰岛素抵抗患者培养淋巴细胞中胰岛素结合减少。
J Clin Endocrinol Metab. 1982 May;54(5):919-30. doi: 10.1210/jcem-54-5-919.
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Impaired growth in Rabson-Mendenhall syndrome: lack of effect of growth hormone and insulin-like growth factor-I.
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Insulin receptor tyrosine kinase activity is abnormal in circulating cells and cultured fibroblasts but normal in transformed lymphocytes from a type A insulin-resistant patient.在一名A型胰岛素抵抗患者的循环细胞和培养的成纤维细胞中,胰岛素受体酪氨酸激酶活性异常,但在其转化淋巴细胞中正常。
J Lab Clin Med. 1988 Jul;112(1):122-32.
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Extreme insulin resistance in ataxia telangiectasia: defect in affinity of insulin receptors.共济失调毛细血管扩张症中的极端胰岛素抵抗:胰岛素受体亲和力缺陷
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Major circadian variations of glucose homeostasis in a patient with Rabson-Mendenhall syndrome and primary insulin resistance due to a mutation (Cys284-->Tyr) in the insulin receptor alpha-subunit.一名患有拉布森-门登霍尔综合征且因胰岛素受体α亚基突变(Cys284→Tyr)导致原发性胰岛素抵抗的患者,其葡萄糖稳态存在明显的昼夜节律变化。
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Cell culture studies on patients with extreme insulin resistance. I. Receptor defects on cultured fibroblasts.极端胰岛素抵抗患者的细胞培养研究。I. 培养成纤维细胞的受体缺陷。
J Clin Endocrinol Metab. 1982 Feb;54(2):261-8. doi: 10.1210/jcem-54-2-261.
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Demonstration of a primary (? genetic) defect in insulin receptors in fibroblasts from a patient with the syndrome of insulin resistance and acanthosis nigricans type A.一名患有A型胰岛素抵抗和黑棘皮症综合征患者的成纤维细胞中胰岛素受体原发性(可能为遗传性)缺陷的证明。
J Clin Endocrinol Metab. 1980 Jun;50(6):1139-41. doi: 10.1210/jcem-50-6-1139.

引用本文的文献

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A Novel Mutation in the Gene Causes Severe Insulin Resistance and Rabson-Mendenhall Syndrome in a Paraguayan Patient.一个新的基因突变导致巴拉圭患者严重胰岛素抵抗和 Rabson-Mendenhall 综合征。
Int J Mol Sci. 2024 Mar 8;25(6):3143. doi: 10.3390/ijms25063143.
2
Genetic insulin resistance is a potent regulator of gene expression and proliferation in human iPS cells.遗传性胰岛素抵抗是人类诱导多能干细胞基因表达和增殖的有力调节因子。
Diabetes. 2014 Dec;63(12):4130-42. doi: 10.2337/db14-0109. Epub 2014 Jul 24.
3
Rabson-Mendenhall syndrome: two case reports and a brief review of the literature.
拉布森-门登霍尔综合征:两例病例报告并文献复习。
Odontology. 2010 Feb;98(1):89-96. doi: 10.1007/s10266-009-0106-7. Epub 2010 Feb 16.
4
Rabson-Mendenhall syndrome: medullary sponge kidney, a new component.拉布森-门登霍尔综合征:髓质海绵肾,一个新组分。
Pediatr Nephrol. 2007 Dec;22(12):2141-4. doi: 10.1007/s00467-007-0600-5. Epub 2007 Sep 12.
5
An in-frame insertion in exon 3 and a nonsense mutation in exon 2 of the insulin receptor gene associated with severe insulin resistance in a patient with Rabson-Mendenhall syndrome.一名患有拉布森-门登霍尔综合征的患者,其胰岛素受体基因外显子3存在框内插入,外显子2存在无义突变,这些与严重胰岛素抵抗相关。
Diabetologia. 1993 Nov;36(11):1168-74. doi: 10.1007/BF00401062.
6
Insulin receptor degradation is accelerated in cultured lymphocytes from patients with genetic syndromes of extreme insulin resistance.在患有极端胰岛素抵抗遗传综合征患者的培养淋巴细胞中,胰岛素受体降解加速。
J Clin Invest. 1984 Oct;74(4):1366-74. doi: 10.1172/JCI111547.
7
Analysis of insulin receptors on human lymphoblastoid cell lines by flow cytometry.
Diabetologia. 1984 Jul;27 Suppl:118-20. doi: 10.1007/BF00275665.
8
Insulin receptor biosynthesis in cultured lymphocytes from insulin-resistant patients.胰岛素抵抗患者培养淋巴细胞中的胰岛素受体生物合成
J Clin Invest. 1985 Dec;76(6):2355-61. doi: 10.1172/JCI112247.
9
Defective processing of insulin-receptor precursor in cultured lymphocytes from a patient with extreme insulin resistance.一名极度胰岛素抵抗患者培养淋巴细胞中胰岛素受体前体加工缺陷。
J Clin Invest. 1988 Jun;81(6):2020-2. doi: 10.1172/JCI113553.
10
Fibroblasts from a leprechaun patient have defects in insulin binding and insulin receptor autophosphorylation.妖精貌综合征患者的成纤维细胞在胰岛素结合和胰岛素受体自身磷酸化方面存在缺陷。
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