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Tubuler Disorder

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https://www.readbyqxmd.com/read/28636607/three-dimensional-cell-shapes-and-arrangements-in-human-sweat-glands-as-revealed-by-whole-mount-immunostaining
#1
Ryuichiro Kurata, Sugiko Futaki, Itsuko Nakano, Fumitaka Fujita, Atsushi Tanemura, Hiroyuki Murota, Ichiro Katayama, Fumihiro Okada, Kiyotoshi Sekiguchi
Because sweat secretion is facilitated by mechanical contraction of sweat gland structures, understanding their structure-function relationship could lead to more effective treatments for patients with sweat gland disorders such as heat stroke. Conventional histological studies have shown that sweat glands are three-dimensionally coiled tubular structures consisting of ducts and secretory portions, although their detailed structural anatomy remains unclear. To better understand the details of the three-dimensional (3D) coiled structures of sweat glands, a whole-mount staining method was employed to visualize 3D coiled gland structures with sweat gland markers for ductal luminal, ductal basal, secretory luminal, and myoepithelial cells...
2017: PloS One
https://www.readbyqxmd.com/read/28598689/mathematical-rationalization-for-the-renal-tubular-transport-revised-concepts
#2
Roberto Mioni, Alessandra Marega, Giulio Romano, Domenico Montanaro
The current emphasis on kinetics and in situ control of molecular exchanges, across the tubular membrane, has not been paralleled by corresponding improvements in our understanding of tubular behaviour at the macroscopic level of classical physiology. In this paper, we propose a mathematical rationalization of macroscopic tubular transport by means of a principal transport equation, originating from the law of mass action between substrate and carrier. The other equations, derived from the main one, demonstrate the possibility of distinguishing between transporters with low affinity and high capacity and transporters with high affinity and low capacity...
June 9, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28551791/mitochondrial-mechanosensor-microdomains-in-cardiovascular-disorders
#3
Michele Miragoli, Aderville Cabassi
The cardiomyocytes populating the 'working myocardium' are highly organized and such organization ranges from macroscale (e.g. the geometrical rod shape) to microscale (dyad/t-tubules) domains. This meticulous level of organization is imperative for assuring the normal and physiological pump-function of the heart. In the pathological cardiac tissue, the domains-related architecture is partially lost, resulting in morphological, electrical and metabolic remodeling and promoting cardiovascular diseases including heart failure and arrhythmias...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28530841/the-increased-expression-of-toll-like-receptor-4-in-renal-and-skin-lesions-in-lupus-erythematosus
#4
Nesrine Elloumi, Raouia Fakhfakh, Lobna Ayadi, Khadija Sellami, Olfa Abida, Mariem Ben Jmaa, Tahya Sellami, Khawla Kammoun, Hatem Masmoudi
Toll-like receptor 4 (TLR-4), a bacterial lipopolysaccharide sensor, is an innate immunity essential modulator. It is expressed on both immune and non-immune cells and may contribute to the cutaneous and renal manifestations during lupus erythematosus (LE). Our purpose is to evaluate TLR-4 expression and analyzing its role in lupus nephritis (LN) and chronic cutaneous lupus erythematosus (CLE) pathogenesis. TLR-4 immunohistochemical staining was performed on 30 LN renal biopsies compared with 11 healthy renal tissues and 30 skin biopsies from CLE patients compared with 15 normal individuals...
May 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28495475/ameliorative-effects-of-achillea-millefolium-inflorescences-alcoholic-extract-against-nicotine-induced-reproductive-failure-in-rat
#5
M Hallaj Salahipour, Sh Hasanzadeh, H Malekinejad
BACKGROUND: Nicotine (Nic) is a major risk factor in the development of functional disorders of male reproductive system. Achillea millefolium; is highly regarded for medicinal activities, due to its antioxidant and anti-inflammatory properties. This study was carried out to evaluate whether Achillea millefolium (Achm) inflorescences alcoholic extract could serve as a protective agent in male reproductive male failures during Nic exposure in a rat model. METHODS: Twenty-five adult male Wistar rats were categorized into the five groups...
May 8, 2017: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/28490965/protective-effects-of-fennel-oil-extract-against-sodium-valproate-induced-hepatorenal-damage-in-albino-rats
#6
Wael M Al-Amoudi
Foeniculum vulgare (Apiaceae) is commonly known as fennel. This herb is well-known worldwide and traditionally used as curative herbal therapy for the treatment of epileptic disease, seizurescarminative, digestive, lactogogue, diuretic, treating respiratory and gastrointestinal disorders. The aim of present study is to investigate the possible effect of fennel oil against the toxicity of Sodium-Valproic (SVP) in albino rats. In order to assess the protection of fennel oil on SVP induced hepato- and nephro-toxicity, male albino rats were treated with 1 ml/kg b...
May 2017: Saudi Journal of Biological Sciences
https://www.readbyqxmd.com/read/28486600/sclt1-deficiency-causes-cystic-kidney-by-activating-erk-and-stat3-signaling
#7
Jianshuang Li, Di Lu, Huadie Liu, Bart O Williams, Paul A Overbeek, Brendan Lee, Ling Zheng, Tao Yang
Ciliopathies form a group of inherited disorders sharing several clinical manifestations due to abnormal cilia formation or function, and few treatments have been successful against these disorders. Here, we report a mouse model with mutated Sclt1 gene, which encodes a centriole distal appendage protein important for ciliogenesis. SCLT1 mutations were associated with the oral-facial-digital syndrome (OFD), an autosomal recessive ciliopathy. The Sclt1-/- mice exhibit typical ciliopathy phenotypes, including cystic kidney, cleft palate, and polydactyly...
May 9, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28473402/post-myocardial-infarction-t-tubules-form-enlarged-branched-structures-with-dysregulation-of-junctophilin-2-and-bridging-integrator-1-bin-1
#8
Christian Pinali, Nadim Malik, J Bernard Davenport, Laurence J Allan, Lucy Murfitt, Mohammad M Iqbal, Mark R Boyett, Elizabeth J Wright, Rachel Walker, Yu Zhang, Halina Dobryznski, Cathy M Holt, Ashraf Kitmitto
BACKGROUND: Heart failure is a common secondary complication following a myocardial infarction (MI), characterized by impaired cardiac contraction and t-tubule (t-t) loss. However, post-MI nano-scale morphological changes to the remaining t-ts are poorly understood. METHOD AND RESULTS: We utilized a porcine model of MI, using a nonlethal microembolization method to generate controlled microinfarcts. Using serial block face scanning electron microscopy, we report that post-MI, after mild left-ventricular dysfunction has developed, t-ts are not only lost in the peri-infarct region, but also the remnant t-ts form enlarged, highly branched disordered structures, containing a dense intricate inner membrane...
May 4, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28456420/the-evolution-of-outer-retinal-tubulation-a-neurodegeneration-and-gliosis-prominent-in-macular-diseases
#9
Rosa Dolz-Marco, Katie M Litts, Anna C S Tan, K Bailey Freund, Christine A Curcio
PURPOSE: To document outer retinal tubulation (ORT) formation in advanced retinal disorders. DESIGN: Retrospective, observational study. PARTICIPANTS: Consecutive cases with retinal diseases showing outer retinal disruption and atrophy of the retinal pigment epithelium (RPE) associated with ORT on spectral-domain (SD) optical coherence tomography (OCT) at the final available visit. METHODS: Cross-sectional SD OCT scans showing ORT at the last available visit were compared with eye-tracked baseline scans...
April 26, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28446726/low-concentration-of-bpa-induces-mice-spermatocytes-apoptosis-via-gpr30
#10
Chaoliang Wang, Jianxiang Zhang, Qi Li, Tianbiao Zhang, Zishi Deng, Jing Lian, Donghui Jia, Rui Li, Tao Zheng, Xiaoju Ding, Fan Yang, Chao Ma, Rui Wang, Weixing Zhang, Jian Guo Wen
Bisphenol A (BPA) acts as xenoestrogen and has a great impact on disorders of human reproductive system. However, the mechanism through which BPA can affect human testicular function remains to be indentified. GPR30 is a novel membrane estrogen receptor with high-affinity and low-capacity binding to estrogens. We demonstrated that estrogen receptor α (ERα), estrogen receptor β (ERβ) as well as GPR30 are expressed in mouse spermatocyte-derived GC-2 cells using Real-time PCR. We treated the cells with different doses of BPA and found that even low doses of BPA can inhibit GC-2 cell growth using MTT assay...
April 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409351/inflammation-and-fibrosis-in-polycystic-kidney-disease
#11
Cheng Jack Song, Kurt A Zimmerman, Scott J Henke, Bradley K Yoder
Polycystic kidney disease (PKD) is a commonly inherited disorder characterized by cyst formation and fibrosis (Wilson, N Engl J Med 350:151-164, 2004) and is caused by mutations in cilia or cilia-related proteins, such as polycystin 1 or 2 (Oh and Katsanis, Development 139:443-448, 2012; Kotsis et al., Nephrol Dial Transplant 28:518-526, 2013). A major pathological feature of PKD is the development of interstitial inflammation and fibrosis with an associated accumulation of inflammatory cells (Grantham, N Engl J Med 359:1477-1485, 2008; Zeier et al...
2017: Results and Problems in Cell Differentiation
https://www.readbyqxmd.com/read/28409340/drosophila-malpighian-tubules-a-model-for-understanding-kidney-development-function-and-disease
#12
Naveen Kumar Gautam, Puja Verma, Madhu G Tapadia
The Malpighian tubules of insects are structurally simple but functionally important organs, and their integrity is important for the normal excretory process. They are functional analogs of human kidneys which are important physiological organs as they maintain water and electrolyte balance in the blood and simultaneously help the body to get rid of waste and toxic products after various metabolic activities. In addition, it receives early indications of insults to the body such as immune challenge and other toxic components and is essential for sustaining life...
2017: Results and Problems in Cell Differentiation
https://www.readbyqxmd.com/read/28404591/intravital-imaging-of-the-kidney-in-a-rat-model-of-salt-sensitive-hypertension
#13
Bradley T Endres, Ruben M Sandoval, George J Rhodes, Silvia B Campos-Bilderback, Malgorzata M Kamocka, Christopher McDermott-Roe, Alexander Staruschenko, Bruce A Molitoris, Aron M Geurts, Oleg Palygin
Hypertension is one of the most prevalent diseases worldwide, and a major risk factor for renal failure and cardiovascular disease. The role of albuminuria, a common feature of hypertension and robust predictor of cardiorenal disorders, remains incompletely understood. The goal of this study was to investigate the mechanisms leading to albuminuria in the kidney of a rat model of hypertension, the Dahl salt-sensitive (SS) rat. To determine the relative contributions of the glomerulus and proximal tubule (PT) to albuminuria, we applied intravital two-photon-based imaging to investigate the complex renal physiological changes that occur during salt-induced hypertension...
April 12, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28365214/in-vivo-localization-of-the-neuronal-ceroid-lipofuscinosis-proteins-cln3-and-cln7-at-endogenous-expression-levels
#14
Alamin Mohammed, Megan B O'Hare, Alice Warley, Guy Tear, Richard I Tuxworth
The neuronal ceroid lipofuscinoses are a group of recessively inherited, childhood-onset neurodegenerative conditions. Several forms are caused by mutations in genes encoding putative lysosomal membrane proteins. Studies of the cell biology underpinning these disorders are hampered by the poor antigenicity of the membrane proteins, which makes visualization of the endogenous proteins difficult. We have used Drosophila to generate knock-in YFP-fusions for two of the NCL membrane proteins: CLN7 and CLN3. The YFP-fusions are expressed at endogenous levels and the proteins can be visualized live without the need for overexpression...
July 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28341694/30-years-of-the-mineralocorticoid-receptor-mineralocorticoid-receptor-and-nacl-transport-mechanisms-in-the-renal-distal-nephron
#15
REVIEW
Shigeru Shibata
A key role of aldosterone and mineralocorticoid receptor is to regulate fluid volume and K(+) homeostasis in the body by acting on the renal distal nephron. Global responses of the kidney to elevated aldosterone levels are determined by the coordinate action of different constituent tubule cells, including principal cells, intercalated cells and distal convoluted tubule cells. Recent studies on genetic mutations causing aldosterone overproduction have identified the molecules involved in aldosterone biosynthesis in the adrenal gland, and there is also increasing evidence for mechanisms and signaling pathways regulating the balance between renal NaCl reabsorption and K(+) secretion, the two major effects of aldosterone...
July 2017: Journal of Endocrinology
https://www.readbyqxmd.com/read/28339935/nephrotoxicity-of-anticancer-treatment
#16
Jolanta Malyszko, Klaudia Kozlowska, Leszek Kozlowski, Jacek Malyszko
Severe adverse systemic drug events occur commonly as a result of treatment of cancer patients. Nephrotoxicity of chemotherapeutic agents remains a significant complication limiting the efficacy of the treatment. A variety of renal disease and electrolyte disorders can result from the drugs that are used to treat malignant disease. The kidneys are a major elimination pathway for many antineoplastic drugs and their metabolites. Tumour lysis syndrome, an emergency in haematooncology, occurs most often after the initiation of cytotoxic therapy in patients with high-grade lymphomas and acute lymphoblastic leukaemia...
June 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28339861/constitutive-luteinizing-hormone-receptor-signaling-causes-sexual-dysfunction-and-leydig-cell-adenomas-in-male-mice1
#17
Lan Hai, Deepak S Hiremath, Marilène Paquet, Prema Narayan
The luteinizing hormone receptor (LHCGR) is necessary for fertility and genetic mutations cause defects in reproductive development and function. Activating mutations in LHCGR cause familial male limited precocious puberty (FMPP). We have previously characterized a mouse model (KiLHRD582G) for FMPP that exhibits the same phenotype of precocious puberty, Leydig cell hyperplasia and elevated testosterone as boys with the disorder. We observed that KiLHRD582G male mice became infertile by 6 months of age although sperm count and motility were normal...
February 17, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/28296922/the-potential-role-of-osteopontin-in-the-maintenance-of-commensal-bacteria-homeostasis-in-the-intestine
#18
Koyu Ito, Akira Nakajima, Yuji Fukushima, Keiichiro Suzuki, Keiko Sakamoto, Yoko Hamazaki, Kouetsu Ogasawara, Nagahiro Minato, Masakazu Hattori
Osteopontin (Opn), a multifunctional extracellular matrix protein, is implicated in the pathogenesis of various inflammatory disorders. Under physiologic conditions, its expression is restricted to certain tissues including bone and kidney tubule. However, cellular activation during disease development induces Opn expression in various immune cells. In this study, using Opn-EGFP knock-in (KI) mice we found that CD8α+ T cells in the intestinal tissues, including Peyer's patch, lamina propria and epithelium, express Opn under steady state conditions...
2017: PloS One
https://www.readbyqxmd.com/read/28288491/effect-of-traditional-treatment-of-diabetes-mellitus-with-xanthosoma-sagittifolium-on-the-male-reproductive-system-of-alloxan-induced-diabetic-wistar-rats
#19
Olayinka A Oridupa, Oluyemisi F Folasire, Adedotun J Owolabi, Oluwasanmi Aina
The management of diabetes mellitus (DM) targets glycemic control as well as prevention or reversal of other complications associated with the metabolic disorder. One of such complications is the infertility which has been traced to oxidative stress and DNA damage caused by DM. In this study, experimentally-induced diabetic male Wistar rats were fed with Xanthosoma sagittifolium (cocoyam) for 14 days and its effect on sperm morphology and characteristics, as well as histology of the testes were assessed. X...
June 2017: Drug Research
https://www.readbyqxmd.com/read/28288174/poor-phenotype-genotype-association-in-a-large-series-of-patients-with-type-iii-bartter-syndrome
#20
Alejandro García Castaño, Gustavo Pérez de Nanclares, Leire Madariaga, Mireia Aguirre, Álvaro Madrid, Sara Chocrón, Inmaculada Nadal, Mercedes Navarro, Elena Lucas, Julia Fijo, Mar Espino, Zilac Espitaletta, Víctor García Nieto, David Barajas de Frutos, Reyner Loza, Guillem Pintos, Luis Castaño, Gema Ariceta
INTRODUCTION: Type III Bartter syndrome (BS) is an autosomal recessive renal tubule disorder caused by loss-of-function mutations in the CLCNKB gene, which encodes the chloride channel protein ClC-Kb. In this study, we carried out a complete clinical and genetic characterization in a cohort of 30 patients, one of the largest series described. By comparing with other published populations, and considering that 80% of our patients presented the p.Ala204Thr Spanish founder mutation presumably associated with a common phenotype, we aimed to test the hypothesis that allelic differences could explain the wide phenotypic variability observed in patients with type III BS...
2017: PloS One
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