Literature DB >> 6339538

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

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

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.

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Year:  1983        PMID: 6339538     DOI: 10.1210/jcem-56-4-856

Source DB:  PubMed          Journal:  J Clin Endocrinol Metab        ISSN: 0021-972X            Impact factor:   5.958


  12 in total

Review 1.  Rabson-Mendenhall syndrome: two case reports and a brief review of the literature.

Authors:  Renuka J Bathi; Sameena Parveen; Sunil Mutalik; Reema Rao
Journal:  Odontology       Date:  2010-02-16       Impact factor: 2.634

2.  Defective processing of insulin-receptor precursor in cultured lymphocytes from a patient with extreme insulin resistance.

Authors:  T Kakehi; A Hisatomi; H Kuzuya; Y Yoshimasa; M Okamoto; K Yamada; H Nishimura; A Kosaki; H Nawata; F Umeda
Journal:  J Clin Invest       Date:  1988-06       Impact factor: 14.808

3.  Fibroblasts from a leprechaun patient have defects in insulin binding and insulin receptor autophosphorylation.

Authors:  J A Maassen; M P Klinkhamer; G C van der Zon; H Sips; W Möller; H M Krans; D Lindhout; F A Beemer
Journal:  Diabetologia       Date:  1988-08       Impact factor: 10.122

4.  A new familial syndrome with impaired function of three related peptide growth factors.

Authors:  H J Hoepffner; M Dreyer; U Reimers; U Schmidt-Preuss; H P Koepp; H W Rüdiger
Journal:  Hum Genet       Date:  1989-10       Impact factor: 4.132

5.  Analysis of insulin receptors on human lymphoblastoid cell lines by flow cytometry.

Authors:  R Maron; S I Taylor; R Jackson; C R Kahn
Journal:  Diabetologia       Date:  1984-07       Impact factor: 10.122

6.  Binding specificity and intramolecular signal transmission of uncleaved insulin proreceptor in transformed lymphocytes from a patient with extreme insulin resistance.

Authors:  T Sasaoka; Y Shigeta; Y Takata; M Sugibayashi; A Hisatomi; M Kobayashi
Journal:  Diabetologia       Date:  1989-06       Impact factor: 10.122

7.  Five mutant alleles of the insulin receptor gene in patients with genetic forms of insulin resistance.

Authors:  T Kadowaki; H Kadowaki; M M Rechler; M Serrano-Rios; J Roth; P Gorden; S I Taylor
Journal:  J Clin Invest       Date:  1990-07       Impact factor: 14.808

8.  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.

Authors:  D Müller-Wieland; E R van der Vorm; R Streicher; W Krone; E Seemanova; M Dreyer; H W Rüdiger; S R Rosipal; J A Maassen
Journal:  Diabetologia       Date:  1993-11       Impact factor: 10.122

9.  Insulin receptor degradation is accelerated in cultured lymphocytes from patients with genetic syndromes of extreme insulin resistance.

Authors:  A McElduff; J A Hedo; S I Taylor; J Roth; P Gorden
Journal:  J Clin Invest       Date:  1984-10       Impact factor: 14.808

10.  Rabson-Mendenhall syndrome: medullary sponge kidney, a new component.

Authors:  Andrew M Harris; Bryan Hall; Vesna M Kriss; John L Fowlkes; Stefan G Kiessling
Journal:  Pediatr Nephrol       Date:  2007-09-12       Impact factor: 3.714

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