• Clinical and Experimental Pediatrics
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• v.62 no.7
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• pp.274-280
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• 2019

Purpose: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. Methods: Twenty-three patients with prepubertal NS treated at Pusan National University Children's Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were positive for mutations of the PTPN11, RAF1, and SOS1 genes. The five genes undetected (FGU) group was negative for PTPN11, RAF1, SOS1, KRAS, and BRAF gene mutations. The influence of genotype was retrospectively analyzed by comparing the growth parameters after GH therapy. Results: The mean chronological age at the start of GH treatment was $5.85{\pm}2.67years$. At the beginning of the GH treatment, the height standard deviation score (SDS), growth velocity (GV), and lower levels of insulin-like growth factor-1 (IGF)-1 levels were not statistically different among the groups. All the 23 NS patients had significantly increased height SDS and serum IGF-1 level during the 3 years of treatment. GV was highest during the first year of treatment. During the 3 years of GH therapy, the PTPN11, RAF1, and SOS1 groups showed less improvement in height SDS, IGF-1 SDS, and GV, and less increase in bone age-to-chronological age ratio than the FGU group. Conclusion: The 3-year GH therapy in the 23 prepubertal patients with NS was effective in improving height SDS, GV, and serum IGF-1 levels. The FGU group showed a better response to recombinant human GH therapy than the PTPN11, RAF1, and SOS1 groups.

• Development and Reproduction
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• v.14 no.1
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• pp.13-19
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• 2010

We developed a novel dicistronic system for the expression of target cDNA sequences in the milk of transgenic animals using goat beta-casein/hGH fusion construct, pGbc5.5hGH (Lee, 2006) and internal ribosome entry site (IRES) sequences of encephalomyocarditis virus (EMCV). Granulocyte colony-stimulating factor (hG-CSF) cDNA was linked to 3' untranslated region of hGH gene in the pGbc5.5hGH via EMCV IRES sequences. Transgenic mice were generated by microinjection and transgene expression was examined in the milk and mammary gland of transgenic mice at 10 days of lactation. Northern blot analysis showed that hGH gene and hG-CSF cDNA were transcribed as a single dicistronic mRNA. The hG-CSF and hGH proteins were independently translated from the dicistronic mRNA and secreted into the milk of transgenic mice. The highest concentration of hG-CSF and hGH in the milk of transgenic mice were $237{\mu}g/m{\ell}$ and $8,990{\mu}g/m{\ell}$, respectively. In contrast, another hG-CSF expression cassette, in which hG-CSF genomic sequences were inserted into a commercial milk-specific expression vector (pBC1), generated a lower level ($91{\mu}g/m{\ell}$) of hG-CSF expression in the milk of transgenic mice. These results demonstrated that the novel pGbc5.5hGH-based dicistronic construct could be useful for an efficient cDNA expression in the milk of transgenic animals.

• Asian-Australasian Journal of Animal Sciences
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• v.7 no.4
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• pp.467-470
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• 1994

Effects of animo acids infusion into the abomasum on plasma growth hormone (GH) concentration were investigated using three wethers of 54 kg of average body weight. Wethers were infused with either 3.25 mmol/kg BW/day of sodium chloride solution (control), 3 mmol/kg BW/day of alanine (Ala), or 3 mmol/kg BW/day of aspartic acid (Asp) continuously for five days through an abomasum cathether in a $3{\times}3$ Latin square desing. On the day of starting infusion (day 0) and day 4 blood samples were collected from a jugular vein every fifteen minutes for six hours after feeding, and their GH concentrations were measured. Blood samples were also collected immediately before starting infusion (day 0), and before feeding of day 1, day 2 and day 4, and their plasma free amino acid concentrations were measured. In the animals infused with Ala, plasma free Ala concentration was increased by Ala infusion and it continued for four days. Plasma GH concentration of these animals increased on day 0, but this phenomenon disappeared on day 4. In the animals infused with Asp, the increase in plasma Asp concentration was observed only on day 1. Plasma GH concentration of these animals was not affected by Asp infusion. These results suggest that continuous Ala infusion stimulates GH secretion for a short period, but the effect would not last long, and that continuous Asp infusion does not affect plasma GH concentration.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.191-196
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• 2005

Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. Results : Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was $0.8{\pm}0.2IU/kg/wk$ in GH treated group. Mean growth velocity was $5.6{\pm}2.0cm/yr$, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were $12.2{\pm}2.7yr$ $10.3{\pm}2.5yr$ $127.5{\pm}10.1cm$ and $-3.1{\pm}1.1$, respectively. In the GH treated group, the mean FAH and SD score of FAH were $146.9{\pm}5.8cm$ and $-2.7{\pm}1.2$, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. Conclusion : GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.

• Korean Journal of Organic Agriculture
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• v.27 no.1
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• pp.65-76
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• 2019

Bacillus genus are found abundantly in various sites and their secondary metabolites were used as potential agents in agriculture, notably plant growth promoting and bio-control. The objective of this study was to develop the culture conditions of GH1-13 strain including higher cell growth, stable endospore-forming and enhancement of potential agents which are related with plant growth promoting and phytopathogen suppression. The optimal carbon and nitrogen sources were determined by glucose and soy bean flour, respectively, then resulted in $7.5{\times}10^9cells/mL$, $6.8{\times}10^9\;endospore\;cells/mL$ and sporulation yield of 90% after 30 h cultivation in 500 L submerged fermenter at $37^{\circ}C$, pH 7.0. Cells and cell-free supernatant of GH1-13 strains showed the potent antifungal activity against phytopathogenic fungi of Colletotrichum gloeosporioides. It was also confirmed that indole-3-acetic acid (IAA) production of GH1-13 strain was greatly increased by addition of 0.3% tryptophan.

• Journal of Korean Neurosurgical Society
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• v.41 no.6
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• pp.367-370
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• 2007

Objective: Pituitary adenomas are common neurological lesions believed to account for 10% to 15% of all primary brain tumors. There can be diagnostic confusion due to discordance of the preoperative endocrine and the postoperative immunohistochemical diagnosis. In this study, the rate of discordance between preoperative and postoperative findings and their clinical implications were investigated. Methods: From March 2005 to March 2006, 26 patients who underwent surgery for a pituitary adenoma were enrolled in this study. The preoperative pituitary hormone level and postoperative immunohistochemical results were compared and analyzed. Results: The median age of the patients was 38 years [range 15-66 years]. The male to female ratio was 8 to 18. The endocrine evaluation showed 16 hormonally-active and 10 hormonally-inactive adenomas. The immunohistochemical findings showed : 13 prolactin-positive, 1 GH-positive, 1 FSH-positive, 8 pleurihormone-positive and 3 stain-negative adenomas. The percentage of discordance observed between the preoperative endocrine and postoperative immunohistochemical diagnosis was 54%. Nine of 10 endocrine non-functioning adenomas showed : 3 PRL positive, 1 GH positive, 2 PRL+GH positive, 1 TSH+FSH positive, 1 FSH+ACTH+PRL positive and 1 FSH+LH+PRL positive adenomas by immunohistochemistry. Three endocrine PRL+GH secreting adenomas showed 2 PRL positive and 1 FSH+GH positive by immunohistochemistry. One endocrine PRL secreting and 1 GH secreting adenoma showed 1 PRL+ TSH positive and 1 GH+PRL positive by immunohistochemistry, respectively. The diagnosis of the other 12 pituitary adenomas showed concordance. Conclusion : The results of this study showed 54% discordance rate between the preoperative endocrine and postoperative immunohistochemical diagnosis for pituitary adenomas.

• Journal of Microbiology and Biotechnology
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• v.31 no.10
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• pp.1438-1445
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• 2021

A bifunctional glycoside hydrolase GH78 from the ascomycete Xylaria polymorpha (XpoGH78) possesses catalytic versatility towards both glycosides and esters, which may be advantageous for the efficient degradation of the plant cell-wall complex that contains both diverse sugar residues and esterified structures. The contribution of XpoGH78 to the conversion of lignocellulosic materials without any chemical pretreatment to release the water-soluble aromatic fragments, carbohydrates, and methanol was studied. The disintegrating effect of enzymatic lignocellulose treatment can be significantly improved by using different kinds of hydrolases and phenoloxidases. The considerable changes in low (3 kDa), medium (30 kDa), and high (> 200 kDa) aromatic fragments were observed after the treatment with XpoGH78 alone or with this potent cocktail. Synergistic conversion of rape straw also resulted in a release of 17.3 mg of total carbohydrates (e.g., arabinose, galactose, glucose, mannose, xylose) per gram of substrate after incubating for 72 h. Moreover, the treatment of rape straw with XpoGH78 led to a marginal methanol release of approximately 17 ㎍/g and improved to 270 ㎍/g by cooperation with the above accessory enzymes. In the case of beech wood conversion, the combined catalysis by XpoGH78 and laccase caused an effect comparable with that of fungal strain X. polymorpha in woody cultures concerning the liberation of aromatic lignocellulose fragments.

• Journal of Interdisciplinary Genomics
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• v.4 no.2
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• pp.35-38
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• 2022

Background: Prader-Willi syndrome (PWS) is a complex genetic disease associated with growth impairment, severe obesity and metabolic dysfunctions. High proportion of PWS patients are born small for gestational age (SGA) than normal children, which also increase the risk of growth impairment and metabolic dysfunction in PWS. We aimed to compare growth outcome and metabolic profiles between SGA and appropriate for gestational age (AGA) PWS patients. Methods: Data of 55 PWS children and adults aged more than 2 years old (32 male and 23 female, age 2-18.8 years) from single center were studied. Only patients who were treated with GH were included. The clinical characteristics and laboratory findings were reviewed retrospectively. Results: Among 55 subjects, 39 had 15q11-13 deletion and 16 had uniparental disomy (UPD). Twenty (36.3%) were born SGA. All patients received GH treatment, and 11 (20%) discontinued GH treatment. Mean age at GH treatment initiation was 2.5 (range 0.3-12.4) years, and mean duration of treatment was 6.3 (range 1.0-11.3) years. Current height-SDS (-0.36 vs -0.16) and BMI-SDS (1.44 vs 1.33) did not differ between AGA and SGA group. Two patients in SGA group, but none in AGA group had diabetes mellitus. Mean glucose level was also higher in SGA group (100.1 vs 114.4 mg/dL). Conclusion: Our report gives an overview of growth profile and metabolic dysfunctions recorded in GH treated PWS patients. Growth profile did not differ between AGA and SGA group. Glucose level was higher in SGA group, so more careful monitoring and prevention for DM will be required in SGA group.

• Animal Bioscience
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• v.34 no.12
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• pp.1886-1894
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• 2021

Objective: Growth hormone (GH) and insulin-like growth factor I (IGF-I) play a critical role in animal growth rates. We aimed to investigate the effect of GH and IGF-I genotypes on body weight (BW), dominance, and gene expression in slow-growing chickens at different ages. Methods: A total of 613 Korat chickens (KRs) were bred and divided into three groups by genotype - A1A1, A1A3, and A3A3 for GH and AA, AC, and CC for IGF-I. Chickens were weighed every two weeks, and liver and breast muscle tissues were collected at 10 weeks of age. Genetic parameters of KRs were estimated using ASReml software. The GH and IGF-I mRNA levels were measured by quantitative polymerase chain reaction. Significant differences between traits were analyzed using the generalized linear model. Results: A significant effect of GH genotypes on BW was found at most ages, and the A1A1 genotype had the highest value of BW. Compared with the A3A3 genotype, the A1A1 and A1A3 genotypes showed a higher dominance effect at 0 and 2 weeks, and genotype A1A1 had the highest value of dominance at 8 weeks of age. A difference in GH mRNA levels between genotypes was detected in breast muscle at 6 weeks and in the liver tissue at 2 weeks. In the case of IGF-I gene, the AA genotype had the highest BW at the beginning of life. Significant differences in BW dominance were found at 2 weeks. However, IGF-I mRNA levels were not different among genotypes in both breast muscles and liver tissues. Conclusion: Our results revealed that GH and IGF-I influence growth, but may not be involved in heterosis. GH can be used as a marker gene in selection programs for growth because the homozygous genotype (A1A1) had the highest BW at all ages. The IGF-I is not a useful marker gene for selection programs.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.5
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• pp.754-768
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• 2008

The past decades have seen enormous advances in our understanding of how GH acts. GH is a pituitary-derived polypeptide hormone that has diverse physiological effects including the regulation of bone growth, carbohydrate and lipid metabolism. The effects of GH are mediated directly and indirectly through IGF-I. In addition, GH stimulates the hepatic production of ALS. In postnatal life, IGF-I and -II circulate as 150 kDa ternary complexes consisting of one molecule each of IGFBP-3 or IGFBP-5, IGF-I or IGF-II and ALS. It is now known that ALS increases significantly the half-lives of the IGFs, IGFBP-3 and -5, and therefore is responsible for maintaining a circulating reservoir for each of these proteins.