scholarly journals Severe growth failure associated with a novel heterozygous nonsense mutation in the GHR transmembrane domain leading to elevated growth hormone binding protein

2020 ◽  
Vol 92 (4) ◽  
pp. 331-337 ◽  
Author(s):  
Ankur Rughani ◽  
Dongsheng Zhang ◽  
Kanimozhi Vairamani ◽  
Andrew Dauber ◽  
Vivian Hwa ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Nonsense Mutation ◽  
Transmembrane Domain ◽  
Binding Protein ◽  
Growth Failure ◽  
Hormone Binding ◽  
Growth Hormone Binding Protein ◽  
Hormone Binding Protein ◽  
Severe Growth

Growth hormone-binding protein levels: Studies of children with short stature

Metabolism ◽  
1994 ◽  
Vol 43 (3) ◽  
pp. 357-359 ◽  
Author(s):  
Nelly Mauras ◽  
Lena M.S. Carlsson ◽  
Suzanne Murphy ◽  
Thomas J. Merimee
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Binding Protein ◽  
Hormone Binding ◽  
Protein Levels ◽  
Growth Hormone Binding Protein ◽  
Hormone Binding Protein

Comparison between the human serum growth hormone-binding protein and the water-soluble growth hormone-binding site released from IM-9 lymphocytes

1993 ◽  
Vol 129 (6) ◽  
pp. 559-564 ◽  
Author(s):  
Guy Massa ◽  
Mapoko Ilondo ◽  
Magda Vanderschueren-Lodeweyckx
Keyword(s):  
Growth Hormone ◽  
Human Serum ◽  
Binding Site ◽  
Binding Protein ◽  
Water Soluble ◽  
Hormone Binding ◽  
Serum Growth Hormone ◽  
Gh Receptor ◽  
Growth Hormone Binding Protein ◽  
Hormone Binding Protein

The characteristics of the human serum growth hormone-binding protein (GHBP) were compared with those of a water-soluble GH-binding site prepared by incubating cultured IM-9 lymphocytes in assay buffer with 25 mmol/l iodoacetamide. High-performance liquid chromatography gel filtration of the water-soluble GH-binding site incubated with 125I-labeled human GH ([125I]hGH) revealed a large peak of bound [125I]hGH eluting at the same position as the peak of [125I]hGH bound to the GHBP in serum. The estimated Mr of the peak was 120 000, presumably representing one [125I]hGH bound to two binding sites. The binding specificities of the serum GHBP, the water-soluble GH-binding site and the GH receptor on IM-9 lymphocytes were identical. The binding affinities for 22 000 hGH and for 20 000 hGH of the serum GHBP were similar to the binding affinity of the water-soluble GH-binding site but lower than those of the cellular GH receptor. These findings show that the characteristics of the serum GHBP are comparable to those of the water-soluble GH-binding site released from IM-9 cells and support the hypothesis that in man the serum GHBP is produced by proteolytic cleavage of the cellular GH receptor.

Growth hormone binding protein and growth hormone availability in acromegalic patients treated with long-acting octreotide (Sandostatin-LAR)

1997 ◽  
Vol 136 (1) ◽  
pp. 61-66 ◽  
Author(s):  
Sanne Fisker ◽  
Andreas Kaal ◽  
Marcella Montini ◽  
Alberto Pedroncelli ◽  
Giorgio Pagani ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Binding Protein ◽  
Hormone Binding ◽  
Age And Gender ◽  
Growth Hormone Binding Protein ◽  
Igf I ◽  
Octreotide Treatment ◽  
Hormone Binding Protein ◽  
And Gender ◽  
Long Acting

Abstract Objective: In the medical treatment of acromegaly different factors are influencial; among these the impact on growth hormone binding protein (GHBP) has not been clarified. Design: Twenty acromegalic patients and nineteen age- and gender-matched normal subjects participated in this study. The patients were treated for 21 months with depot long-acting microsphere-enclosed octreotide (Sandostatin-LAR). Previously, all the patients were treated s.c. with octreotide t.i.d. After a 2-week wash-out period (baseline) the patients received the first i.m. injection of the long-acting octreotide. The first two injections were administered at 60-day intervals; thereafter the injections were at 28-day intervals. Methods: The levels of GHBP, complexed GHBP, growth hormone (GH) and insulin-like growth factor-I (IGF-I) were determined in fasting serum samples. Results: In the 2-week wash-out period GHBP levels decreased from 1·13 ± 0·17 to 0·92 ± 0·15 nmol/l (P < 0·05). During the 21-months treatment, GHBP increased again to 1·10 ± 0·16 nmol/l. In the age- and gender-matched control group GHBP levels were significantly higher at all times (1·95 ± 0·21 nmol/l, P(all) < 0·02). Mean levels of 8-h GH decreased from 12·6 ± 2·58 μg/l at baseline to 1·97 ± 0·20 μg/l after 21 months of treatment (P < 0·05). Mean 8-h GH levels were unchanged during long-acting octreotide treatment compared with levels during s.c. treatment (1·97 ± 0·20 μg/l and 1·90 ± 0·20 μg/l respectively). In fasting blood samples GH-complexed GHBP ranged from 13·8 ± 2·4% (9 months) to 25·4 ± 4·5% (baseline) of total GHBP. Serum IGF-I increased from 367 ± 45 to 764 ± 80 μg/l (P < 0·05) during the 2-week wash-out period and decreased to 290 ± 35 μg/l (P < 0·05) after 21 months of treatment with long-acting octreotide. IGF-I levels after 21 months were significantly lower than during s.c. octreotide treatment (P < 0·05). Conclusion: Serum GHBP levels are similar during treatment with long-acting octreotide as compared with regular octreotide. Furthermore, significant changes in GHBP can occur within 2 weeks. Finally, in addition to the lowering effect on GH levels, the induced increase in GHBP levels may imply a further advantage in octreotide treatment of acromegaly, circulating GH bound to GHBP may less readily reach the tissues. European Journal of Endocrinology 136 61–66

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