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https://www.readbyqxmd.com/read/29649050/temporal-bone-histopathology-in-cockayne-syndrome
#1
Ophir Handzel, Joseph B Nadol
: Cockayne syndrome (CS) is a rare autosomal recessive syndrome resulting in defective DNA repair. Its features include cachectic dwarfism, hearing loss, skin hypersensitivity to sunlight, premature aging, and dementia. Presented is a right temporal bone of a patient who died at the age of 29 years. The clinical course was compatible with type 1 CS, the classical form. Homozygous missense variant in the ERCC6 gene (Excision Repair Cross-Complementation group 6) was found, compatible with CS complementation group B...
April 11, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29625454/white-matter-abnormalities-and-correlation-with-severity-in-normal-tension-glaucoma-a-whole-brain-atlas-based-diffusion-tensor-study
#2
Rong Wang, Zuohua Tang, Xinghuai Sun, Lingjie Wu, Jie Wang, Yufeng Zhong, Zebin Xiao
Purpose: To detect injury of whole brain white matter (WM) in normal tension glaucoma (NTG) patients by using diffusion tensor imaging (DTI) and to analyze the correlations between DTI parameters and glaucoma indices. Methods: Twenty mild, 17 moderate, and 18 severe NTG patients as well as 25 normal subjects were enrolled in this study. Atlas-based diffusion tensor analysis was performed to measure the fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD)...
March 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29618970/corticotropin-releasing-factor-receptors-modulate-oxytocin-release-in-the-dorsolateral-bed-nucleus-of-the-stria-terminalis-bnst-in-male-rats
#3
Daisy Martinon, Joanna Dabrowska
The neuropeptide oxytocin (OT) plays an important role in the regulation of social and anxiety-like behavior. Our previous studies have shown that OT neurons send projections from the hypothalamus to the dorsolateral bed nucleus of the stria terminalis (BNSTdl ), a forebrain region critically involved in the modulation of anxiety-like behavior. Importantly, these OT terminals in the BNSTdl express presynaptic corticotropin releasing factor (CRF) receptor type 2 (CRFR2). This suggests that CRFR2 might be involved in the modulation of OT release...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29596647/male-reproductive-behavior-sensory-signaling-in-the-brain-of-low-performing-domestic-rams
#4
Brenda M Alexander
Rams are selected for genetic traits a producer desires to propagate in his flock. Even though practically all sheep are naturally bred, rams are rarely evaluated for expression of sexual interest or behavior. Research at the United States Sheep Experiment Station reported that the proportion of rams with limited interest in ewes was nearly 30% of the total number of breeding rams. Breeding soundness tested rams with low sexual-interest sire less than 16% of the lamb crop. While producers recognize the problem, their usual solution is to increase the number of rams in breeding flocks decreasing the risk of open ewes...
March 27, 2018: Journal of Animal Science
https://www.readbyqxmd.com/read/29569009/paraventricular-hypothalamic-and-amygdalar-crf-neurons-synapse-in-the-external-globus-pallidus
#5
Albert J Hunt, Rajan Dasgupta, Shivakumar Rajamanickam, Zhiying Jiang, Michael Beierlein, C Savio Chan, Nicholas J Justice
Stress evokes directed movement to escape or hide from potential danger. Corticotropin-releasing factor (CRF) neurons are highly activated by stress; however, it remains unclear how this activity participates in stress-evoked movement. The external globus pallidus (GPe) expresses high levels of the primary receptor for CRF, CRFR1, suggesting the GPe may serve as an entry point for stress-relevant information to reach basal ganglia circuits, which ultimately gate motor output. Indeed, projections from CRF neurons are present within the GPe, making direct contact with CRFR1-positive neurons...
March 23, 2018: Brain Structure & Function
https://www.readbyqxmd.com/read/29560385/c-fos-mapping-of-brain-regions-activated-by-multi-modal-and-electric-foot-shock-stress
#6
Xiaoxiao Lin, Christy A Itoga, Sharif Taha, Ming H Li, Ryan Chen, Kirolos Sami, Fulvia Berton, Walter Francesconi, Xiangmin Xu
Real-world stressors are complex and multimodal, involving physical, psychological, and social dimensions. However, the brain networks that mediate stress responses to these stimuli need to be further studied. We used c-Fos mapping in mice to characterize brain circuits activated by exposure to a single episode of multimodal stress (MMS), and compared these to circuits activated by electric foot shocks (EFS). We focused on characterizing c-Fos activity in stress-relevant brain regions including the paraventricular nucleus (PVN) of the hypothalamus and the bed nucleus of the stria terminalis (BNST)...
February 2018: Neurobiology of Stress
https://www.readbyqxmd.com/read/29551423/differential-localizations-of-the-myo-inositol-transporters-hmit-and-smit1-in-the-cochlear-stria-vascularis
#7
Midori Edamatsu, Yasuhiro Kondo, Motonori Ando
The cochlear stria vascularis produces endolymph and thereby plays an active role in inner ear homeostasis. We recently reported that the H+ /myo-inositol cotransporter (HMIT) gene is expressed in the stria vascularis. Here, we examined the protein localization of HMIT and Na+ /myo-inositol cotransporter 1 (SMIT1) in the stria vascularis by immunohistochemistry. HMIT and SMIT1 were detected in the lateral wall of the cochlear duct. HMIT was widely detected throughout the stria vascularis, while SMIT1 was enriched in the strial basal cells...
March 15, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29501514/anatomy-of-the-limbic-white-matter-tracts-as-revealed-by-fiber-dissection-and-tractography
#8
Raluca Pascalau, Roxana Popa Stănilă, Silviu Sfrângeu, Bianca Szabo
BACKGROUND: The limbic tracts are involved in crucial cerebral functions such as memory, emotion and behavior. The complex architecture of the limbic circuit makes it harder to approach compared to other white matter networks. Our study aims to describe the three-dimensional anatomy of the limbic white matter by use of two complementary study methods, namely ex vivo fiber dissection and in vivo MRI based tractography. METHODS: Three fiber dissection protocols were performed using blunt wooden instruments and a surgical microscope on formalin fixed brains prepared according to the Klingler method...
February 28, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29483934/two-predominant-mups-obp3-and-mup13-are-male-pheromones-in-rats
#9
Xiao Guo, Huifen Guo, Lei Zhao, Yao-Hua Zhang, Jian-Xu Zhang
Background: In rats, urine-borne male pheromones comprise organic volatile compounds and major urinary proteins (MUPs). A number of volatile pheromones have been reported, but no MUP pheromones have been identified in rat urine. Results: We used sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE), isoelectric focusing electrophoresis (IEF), nano-liquid chromatography-tandem mass spectrometry (nLC-MS/MS) after in gel digestion of the proteins and quantitative real-time PCR (qRT - PCR) and showed that the levels of two MUPs, odorant - binding protein 3 (OBP3) (i...
2018: Frontiers in Zoology
https://www.readbyqxmd.com/read/29466997/hearing-impairment-in-melas-new-prospective-in-clinical-use-of-microrna-a-systematic-review
#10
REVIEW
Arianna Di Stadio, Valentina Pegoraro, Laura Giaretta, Laura Dipietro, Roberta Marozzo, Corrado Angelini
AIM: To evaluate the feasibility of microRNAs (miR) in clinical use to fill in the gap of current methodology commonly used to test hearing impairment in MELAS patients. MATERIAL AND METHOD: A literature review was performed using the following keywords, i.e., MELAS, Hearing Loss, Hearing Impairment, Temporal Bone, Otoacustic Emission (OTOAE), Auditory Brain Response (ABR), and microRNA. We reviewed the literature and focused on the aspect of the temporal bone, the results of electrophysiological tests in human clinical studies, and the use of miR for detecting lesions in the cochlea in patients with MELAS...
February 21, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29458150/pink1-protects-auditory-hair-cells-and-spiral-ganglion-neurons-from-cisplatin-induced-ototoxicity-via-inducing-autophagy-and-inhibiting-jnk-signaling-pathway
#11
Qianqian Yang, Gaoying Sun, Haiyan Yin, Hongrui Li, Zhixin Cao, Jinghan Wang, Meijuan Zhou, Haibo Wang, Jianfeng Li
Phosphatase and tensin homologue (PTEN)-induced putative kinase 1 (PINK1) gene encodes a serine/threonine kinase, which acts as a molecular sensor of mitochondrial health necessary for mitochondrial quality control. The present study was designed to examine whether PINK1 expressed in C57BL/6 murine cochlea and HEI-OC1 cells and, if so, to investigate the possible mechanisms underlying the action of PINK1 in cisplatin-induced death of sensory hair cells (HCs) and spiral ganglion neurons (SGNs) in vitro. The expression pattern of PINK1, formation of parkin particles, and autophagy were determined by immunofluorescent staining...
February 16, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29456958/etiology-of-noise-induced-hearing-loss-nihl-and-its-symptomatic-correlation-with-audiometry-observations-in-type-ii-diabetes
#12
Meena K Yadav, K S Yadav
Type II diabetic mellitus (DM) is a chronic metabolic disease that impairs normal insulin production and glucose transport to the liver and muscles. In the India, about 1-5% population suffer from diabetes or related complication. So there is need to cure this disease. DM chronic auditory complications may include spiral ganglia atrophy, degeneration of the vestibulocochlear nerve myelin sheath, reduction of the number of spiral lamina nerve fibres, and thickening of the capillary walls of the stria vascularis and small arteries...
March 2018: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/29453205/co-deficiency-of-lysosomal-mucolipins-3-and-1-in-cochlear-hair-cells-diminishes-outer-hair-cell-longevity-and-accelerates-age-related-hearing-loss
#13
Teerawat Wiwatpanit, Natalie N Remis, Aisha Ahmad, Yingjie Zhou, John C Clancy, Mary Ann Cheatham, Jaime García-Añoveros
Acquired hearing loss is the predominant neurodegenerative condition associated with aging in humans. Although mutations on several genes are known to cause congenital deafness in newborns, few genes have been implicated in age-related hearing loss (ARHL), perhaps because its cause is likely polygenic. Here, we generated mice lacking lysosomal calcium channel mucolipins 3 and 1 and discovered that both male and female mice suffered a polygenic form of hearing loss. While mucolipin 1 is ubiquitously expressed in all cells, mucolipin 3 is expressed in a small subset of cochlear cells -hair cells (HCs) and marginal cells of the stria vascularis- and very few other cell types...
February 16, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29436578/protective-effect-of-adenovirus-mediated-erythropoietin-expression-on-the-spiral-ganglion-neurons-in-the-rat-inner-ear
#14
Cheng Zhong, Zhendong Jiang, Qiang Guo, Xueyuan Zhang
The aim of the present study was to evaluate the expression of erythropoietin (Epo) and the Epo receptor (Epo‑R) in the spiral ganglion neurons (SGNs) of the rat inner ear, and to assess the effect of Epo adenovirus vector (Ad‑Epo) on the spontaneous apoptosis of SGNs. A total of 60 ears from 30 healthy neonatal (2‑3 days postnatal) Sprague‑Dawley rats were used to examine the expression of Epo in the SGNs. The rats were divided into three groups: The negative control group, the vector control group [infected with a green fluorescent protein expression vector (Ad‑GFP)] and the Ad‑Epo group (infected with Ad‑Epo)...
February 5, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29426600/the-endocochlear-potential-as-an-indicator-of-reticular-lamina-integrity-after-noise-exposure-in-mice
#15
Kevin K Ohlemiller, Tejbeer Kaur, Mark E Warchol, Robert H Withnell
The endocochlear potential (EP) provides part of the electrochemical drive for sound-driven currents through cochlear hair cells. Intense noise exposure (110 dB SPL, 2 h) differentially affects the EP in three inbred mouse strains (C57BL/6 [B6], CBA/J [CBA], BALB/cJ [BALB]) (Ohlemiller and Gagnon, 2007, Hearing Research 224:34-50; Ohlemiller et al., 2011, JARO 12:45-58). At least for mice older than 3 mos, B6 mice are unaffected, CBA mice show temporary EP reduction, and BALB mice may show temporary or permanent EP reduction...
February 1, 2018: Hearing Research
https://www.readbyqxmd.com/read/29424020/activation-patterns-of-vasopressinergic-and-oxytocinergic-brain-regions-following-social-play-exposure-in-juvenile-male-and-female-rats
#16
C J Reppucci, C K Gergely, A H Veenema
Social play is a highly rewarding and motivated behavior predominately displayed by juveniles and expressed by nearly all mammalian species. Prior work suggested that the vasopressin (AVP) and oxytocin (OT) systems can regulate the expression of social play in sex-specific ways. Here we investigated whether there are sex differences in the recruitment of vasopressinergic and oxytocinergic brain regions following social play exposure in juvenile rats. Single-housed rats were allowed to play, in their home cage, with an age- and sex-matched unfamiliar conspecific for 10 min, or received similar handling but no partner...
February 9, 2018: Journal of Neuroendocrinology
https://www.readbyqxmd.com/read/29414029/control-of-cardiovascular-responses-to-stress-by-crf-in-the-bed-nucleus-of-stria-terminalis-is-mediated-by-local-nmda-nnos-sgc-pkg-signaling
#17
Leandro A Oliveira, Lucas Gomes-de-Souza, Ricardo Benini, Carlos C Crestani
The aims of the present study were to assess an interaction of corticotropin-releasing factor (CRF) neurotransmission within the bed nucleus of the stria terminalis (BNST) with local nitrergic signaling, as well as to investigate an involvement of activation of local NMDA glutamate receptor and nitric oxide (NO) signaling in control of cardiovascular responses to acute restraint stress by BNST CRF neurotransmission in rats. We observed that CRF microinjection into the BNST increased local NO release during restraint stress...
January 12, 2018: Psychoneuroendocrinology
https://www.readbyqxmd.com/read/29408589/identification-of-stria-medullaris-fibers-in-the-massa-intermedia-using-diffusion-tensor-imaging
#18
Ryan B Kochanski, Robert Dawe, Mehmet Kocak, Sepehr Sani
BACKGROUND: The massa intermedia (MI) or interthalamic adhesion is an inconsistent band spanning between bilateral medial thalami that is absent in up to 20%-30% of individuals. Little is known of its significance, especially in regard to functional pathways. Probabilistic diffusion tensor imaging (DTI) has recently been used to seed the lateral habenula and define its afferent white matter pathway, the stria medullaris thalami (SM). We sought to determine whether the MI serves as a conduit for crossing of limbic fibers such as the SM...
February 1, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29408468/neuropeptide-y2-receptors-in-anteroventral-bnst-control-remote-fear-memory-depending-on-extinction-training
#19
Dilip Verma, Ramon Tasan, Guenther Sperk, Hans-Christian Pape
The anterior bed nucleus of stria terminalis (BNST) is involved in reinstatement of extinguished fear, and neuropeptide Y2 receptors influence local synaptic signaling. Therefore, we hypothesized that Y2 receptors in anteroventral BNST (BNSTav) interfere with remote fear memory and that previous fear extinction is an important variable. C57BL/6NCrl mice were fear-conditioned, and a Y2 receptor-specific agonist (NPY3-36) or antagonist (JNJ-5207787) was applied in BNSTav before fear retrieval at the following day...
February 2, 2018: Neurobiology of Learning and Memory
https://www.readbyqxmd.com/read/29407458/mechanistic-substrates-of-a-life-history-transition-in-male-prairie-voles-developmental-plasticity-in-affiliation-and-aggression-corresponds-to-nonapeptide-neuronal-function
#20
Aubrey M Kelly, Alexander G Saunders, Alexander G Ophir
Although prairie vole (Microtus ochrogaster) social behavior is well-characterized in adults, surprisingly little is known about the development of social behavior in voles. Further, the overwhelming majority of studies in prairie voles examine social behavior in a reproductive context. Here, we examine developmental plasticity in affiliation and aggression and their underlying neural correlates. Using sexually naïve males, we characterized interactions with an age-matched, novel, same-sex conspecific in four different age groups that span pre-weaning to adulthood...
February 3, 2018: Hormones and Behavior
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