Barth syndrome (BTHS) and dilated cardiomyopathy with ataxia syndrome (DCMA) are biochemically characterized by high levels of 3-methylglutaric acid (MGA) in the urine and plasma of affected patients. Although cardiolipin abnormalities have been observed in these disorders, their pathophysiology is not fully established. We evaluated the effects of MGA administration on redox homeostasis and mitochondrial function in heart, as well as on vascular reactivity in aorta of Wistar rats without cardiolipin genetic deficiency.
View Article and Find Full Text PDFTissue accumulation and high urinary excretion of ethylmalonic acid (EMA) are found in ethylmalonic encephalopathy (EE), an inherited disorder associated with cerebral and cerebellar atrophy whose pathogenesis is poorly established. The in vitro and in vivo effects of EMA on bioenergetics and redox homeostasis were investigated in rat cerebellum. For the in vitro studies, cerebellum preparations were exposed to EMA, whereas intracerebellar injection of EMA was used for the in vivo evaluation.
View Article and Find Full Text PDFAnomalies in the müllerian ducts are congenital alterations with more prevalence than it is imagined, varying from 0.5 to 6.7% in the general population and up to 16.
View Article and Find Full Text PDFMitochondrion
July 2019
We report here the effects of hydrogen sulfide (sulfide), that accumulates in ETHE1 deficiency, in rat cerebellum. Sulfide impaired electron transfer and oxidative phosphorylation. Sulfide also induced mitochondrial swelling, and decreased ΔΨm and calcium retention capacity in cerebellum mitochondria, which were prevented by cyclosporine A (CsA) plus ADP, and ruthenium red, suggesting mitochondrial permeability transition (mPT) induction.
View Article and Find Full Text PDFNon-ketotic hyperglycinemia (NKH) is a severe neurological disorder caused by defects in glycine (GLY) catabolism and characterized by a high cerebrospinal fluid/plasma GLY ratio. Treatment is often ineffective and limited to the control of symptoms and detoxification of GLY. In the present work, we investigated the in vivo effects of GLY intracerebroventricular administration on oxidative stress parameters in rat striatum, cerebral cortex, and hippocampus.
View Article and Find Full Text PDFObjective: This study evaluating the lipid profile in women in post menopause using the hormone therapy (HT) with implants of estradiol and testosterone.
Method: One year prospective cohort with 122 patients separated in group 1, not using HT, group 2 starting the use of HT, and group 3 with prior use of implants of estradiol and testosterone. All patients performed serum dosages of total cholesterol, HDL and triglycerides, in the beginning of the study and after 1 year.
Gynecol Endocrinol
February 2011
Objective: To assess bone mineral density (BMD) in postmenopausal women using estradiol and testosterone hormonal implants comparing to that of patients without hormonal therapy.
Design Of The Study: Sixty-one patients were followed in prospective cohort study separated in Group 1, 34 women using implants and Group 2, 27 women without implants and BMD assessment through Dual energy X-ray absorptiometry was conducted in the beginning of follow-up and after 1 year.
Results: The average lumbar spine BMD in Group 1 was 1.