Generation of the human iPSC lines AKOSi011-A carrying the mutation p.Pro65Ser/p.Asp35T and AKOSi012-A, carrying the mutation p.Tyr231His, derived from FAHN patient fibroblasts.

Fatty acid hydroxylase-associated neurodegeneration (FAHN) is a hereditary neurodegenerative disease caused by mutations in the FA2H gene. Patients show a wide range of neurological symptoms and an abnormal myelination. Here we describe the generation of the human induced pluripotent stem cell (hiPSC) lines AKOSi011-A and AKOSi012-A, derived from FAHN-patient fibroblasts, carrying the compound heterozygous mutation p.Pro65Ser/p.Asp35Tyr and the homozygous mutation p.Tyr231His, respectively. The hiPSC lines were generated using a non-integrating Sendai virus. The obtained hiPSCs show an unobtrusive karyotype, carry the mutations of the original fibroblasts, express pluripotency markers and can differentiate into cells of the three germ layers.

Propolis protects ovarian follicular reserve and maintains the ovary against polycystic ovary syndrome (PCOS) by attenuating degeneration of zona pellucida and fibrous tissue.

To examine the effects of 50 mg/kg and 150 mg/kg of propolis on ovarian folliculogenesis, p53 expression, and serum luteinising hormone (LH) and progesterone (P) levels in polycystic ovary syndrome (PCOS) modeled rats. Twenty-four Wistar female rats were divided into 4 experimental groups: Group 1 (G1, Control), Group 2 (G2, PCOS), Group 3 (G3, PCOS + 50 mg/kg propolis), and Group 4 (G4, PCOS + 150 mg/kg propolis). The PCOS model was induced via the administration of letrozole for 21 days. After 21 days, G3 and G4 received propolis (50 mg/kg or 150 mg/kg) by oral gavage for 10 days. Daily oestrous cycles were assessed to monitor PCOS formation. Histological examinations were carried out using haematoxylin and eosin (H&E) and Masson Trichrome (MT) staining. Ovarian follicles and corpus luteum (CL) structures were investigated. P and LH serum levels were determined by ELISA. A significant increase was observed in the number of cystic follicles in G2 compared to G1 (p < 0.001). Treatment with 50 mg/kg of propolis significantly ameliorated the elevated number of cystic and primary follicles seen in G2 (p < 0.001). Furthermore, G2 demonstrated a significant decrease in the number of CL structures (p < 0.05). Serum LH levels were significantly higher in G4 compared to both G1 and G2 (p < 0.01). No significant change was observed in circulating P levels. No p53 immunoreactivity was observed in any group. Low concentrations of propolis cannot completely improve the hormone profile and p53 expression associated with PCOS; however, these concentrations can control ovarian follicular cell architecture.

Generation of the human iPSC line AKOSi010-A from fibroblasts of a female FAHN patient, carrying the compound heterozygous mutation p.Gly45Arg/p.His319Arg.

Fatty acid hydroxylase-associated neurodegeneration (FAHN) is a rare childhood onset neurodegenerative disease caused by mutations in the FA2H gene. Patients display abnormal myelination, cerebellar atrophy and some have iron deposition in the central nervous system. Here we describe the generation of AKOSi010-A, a human induced pluripotent stem cell (hiPSC) line derived from fibroblasts of a female patient carrying the compound heterozygous p.Gly45Arg/p.His319Arg, using non-integrating Sendai virus. The generated iPSCs express pluripotency markers, can differentiate into cell types of the three germ layers and show a normal karyotype. This cell line displays a unique source to study the pathophysiology of FAHN.

Effects of metformin, letrozole and atorvastatin on inflammation and apoptosis in experimental peritoneal and ovarian endometriosis in the rat.

Endometriosis is a common gynecological hurting disorder in which tissue is similar to the tissue that normally lines the inner layer of the uterus. It often causes fertility problems. Unfortunately, effective treatments are limited. Therefore it's important to explore an imperative and easily accessible treatment to alleviate the probable pathologies and preserve fertility in endometriosis. Consequently, we aimed to investigate the effects of metformin, letrozole, and atorvastatin on inflammation and apoptosis in experimentally induced ovarian and peritoneal endometriosis in rat models. In the present study, 35 rats were randomly divided into five groups. Group 1: sham-operated control group. Group 2: untreated endometriosis group. Group 3: given 100 mg/kg/day of oral metformin. Group 4: given 0.1 mg/kg/day of oral letrozole. Group 5: given 2.5 mg/kg/day of oral atorvastatin. At the end of the 28 days, we examined Ki67, Bax and Bcl-2 immunoexpressions in ovarian and peritoneal tissues, and IL-6, IL-8, and TNF-α levels were evaluated from the peritoneal fluid. All medical treatment groups showed a significant decrease in Ki67 expression. A significant increase in Bax expression was also observed in all samples from all medical treatment groups (other than the untreated endometriosis groups). Further, a significant decrease in Bcl-2 expression was found in all medical treatment groups. IL-6, IL-8, and TNF-α levels were significantly lower in all medical treatment groups than in the endometriosis groups. In conclusion; Metformin, letrozole, and atorvastatin showed apoptosis induction and anti-inflammatory effects on both ovarian and peritoneal endometriosis in experimental models.

Histological and biochemical apoptosis changes of female rats' ovary by Zinc oxide nanoparticles and potential protective effects of l-arginine: An experimental study.

BACKGROUND: This research aims to investigate the adverse effects of ZnO NP on ovarian tissue and the follicular and menstrual cycle and the protective effects of l-arginine on the aforementioned tissues. MATERIAL AND METHODS: 30 rats were divided into five groups. The first group was the control group. The second and fourth groups received 100 mg/kg and 200 mg/kg ZnO NP, respectively. The third and fifth groups received the same doses of ZnO NP as the second and fourth groups, respectively. However, the third and fifth groups received an additional dose of 1.3 gr/kg of LA amino acid. ZnO NP and LA are given intraperitoneal for 21 days. Blood samples from each rat and a part of the ovarium were collected to test for gene expression and histological analysis. RESULTS: Compared to levels of housekeeping gene ß-actine, levels of apoptosis effectors such as Bax, Bcl, Caspase 3, and Caspase 9 were significantly increased in all groups. In groups that received doses of LA (three and five), atretic follicle size was smaller compared to groups that did not receive LA (two and four). In addition, in the third group, the secondary and primordial follicle's generated oocytes were smaller compared with groups two, four, and five. Compared with the control group, all groups experienced morphological degeneration of follicles and tissue. CONCLUSION: ZnO NP has inevitable, morphological, and physiological effects on the ovary and can detrimentally impact the tissue. LA can aid in the regeneration of the tissue and block damage induced by stress and toxicity.