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Absence of mating behaviors in the female dogs neonatally treated with estrogen and progesterone.
Anim Reprod
January 2025
Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois at Urbana-Champaign, Urbana, IL, USA.
This study aimed to develop a non-surgical method to neutralize reproduction in female dogs. Female Beagle puppies, aged 6 days, were treated with pellets designed to release estradiol benzoate (EB; 1.0 mg) and progesterone (P4; 5.
View Article and Find Full Text PDFGene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models.
Sci Transl Med
January 2025
Department of Pediatrics, Washington University in St. Louis, School of Medicine, St. Louis, MO 63110, USA.
Children with neurodegenerative disease often have debilitating gastrointestinal symptoms. We hypothesized that this may be due at least in part to underappreciated degeneration of neurons in the enteric nervous system (ENS), the master regulator of bowel function. To test this hypothesis, we evaluated mouse models of neuronal ceroid lipofuscinosis type 1 and 2 (CLN1 and CLN2 disease, respectively), neurodegenerative lysosomal storage disorders caused by deficiencies in palmitoyl protein thioesterase-1 and tripeptidyl peptidase-1, respectively.
View Article and Find Full Text PDFFunctional evidence that S-nitroso-L-cysteine may be a candidate carotid body neurotransmitter.
Neuropharmacology
March 2025
Department of Pediatrics, Case Western Reserve University, Cleveland, OH, USA; Department of Pharmacology, Case Western Reserve University, Cleveland, OH, USA; Functional Electrical Stimulation Center, Case Western Reserve University, Cleveland, OH, USA.
The primary objective of the present study is to provide further evidence that the endogenous S-nitrosothiol, S-nitroso-L-cysteine (L-CSNO), plays an essential role in signaling the hypoxic ventilatory response (HVR) in rodents. Key findings were that (1) injection of L-CSNO (50 nmol/kg, IV) caused a pronounced increase in frequency of breathing (Freq), tidal volume (TV) and minute ventilation (MV) in naïve C57BL/6 mice, whereas injection of D-CSNO (50 nmol/kg, IV) elicited minimal responses; (2) L-CSNO elicited minor responses in (a) C57BL/6 mice with bilateral carotid sinus nerve transection (CSNX), (b) C57BL/6 mice treated neonatally with capsaicin (CAP) to eliminate small-diameter C-fibers, and (c) C57BL/6 mice receiving continuous infusion of L-CSNO receptor antagonists, S-methyl-L-cysteine and S-ethyl-L-cysteine (L-SMC + L-SEC, both at 5 μmol/kg/min, IV); and (3) injection of S-nitroso-L-glutathione (L-GSNO, 50 nmol/kg, IV) elicited pronounced ventilatory responses that were not inhibited by L-SMC + L-SEC. Subsequent exposure of naïve C57BL/6 mice to a hypoxic gas challenge (HXC; 10% O, 90% N) elicited pronounced increases in Freq, TV and MV that were subject to roll-off.
View Article and Find Full Text PDFImpairment of neuromotor development and cognition associated with histopathological and neurochemical abnormalities in the cerebral cortex and striatum of glutaryl-CoA dehydrogenase deficient mice.
Neurochem Int
December 2024
PPG Ciências Biológicas: Bioquímica, Departamento de Bioquímica, ICBS, UFRGS, Porto Alegre, Brazil; Departamento de Medicina Interna, Faculdade de Medicina, UFRGS, Porto Alegre, Brazil; Serviço de Genética Médica, HCPA, Porto Alegre, Brazil. Electronic address:
Patients with glutaric acidemia type I (GA I) manifest motor and intellectual disabilities whose pathogenesis has been so far poorly explored. Therefore, we evaluated neuromotor and cognitive abilities, as well as histopathological and immunohistochemical features in the cerebral cortex and striatum of glutaryl-CoA dehydrogenase (GCDH) deficient knockout mice (Gcdh), a well-recognized model of GA I. The effects of a single intracerebroventricular glutaric acid (GA) injection in one-day-old pups on the same neurobehavioral and histopathological/immunohistochemical endpoints were also investigated.
View Article and Find Full Text PDFCerebral palsy in Australia: birth prevalence, 1995-2016, and differences by residential remoteness: a population-based register study.
Med J Aust
November 2024
Cerebral Palsy Alliance Research Institute, the University of Sydney, Sydney, NSW.
Objective: To examine recent changes in the birth prevalence of cerebral palsy in Australia; to examine the functional mobility of children with cerebral palsy by residential remoteness.
Study Design: Population-based register study; analysis of Australian Cerebral Palsy Register (ACPR) data.
Setting, Participants: Children with cerebral palsy born in Australia, 1995-2016, and included in the ACPR at the time of the most recent state/territory data provision (31 July 2022).
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