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Tubuler Disorder

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https://www.readbyqxmd.com/read/29743587/three-dimensional-tubule-formation-assay-as-therapeutic-screening-model-for-ocular-microvascular-disorders
#1
Mahnaz Shariatzadeh, Maarten M Brandt, Caroline Cheng, Josianne C Ten Berge, Aniki Rothova, Pieter J M Leenen, Willem A Dik
PURPOSE: This study is aimed to adapt a three-dimensional (3-D) in vitro angiogenesis model to the ophthalmology field using retinal endothelial cells (REC). This system is applied to assess the angiogenic capacity of aqueous humor (AH) from patients with ocular disorders, and to test the effect of VEGF inhibitor (aflibercept) on induced angiogenesis. METHODS: Human REC and umbilical vein endothelial cells (HUVEC) and pericytes were co-cultured in a gel matrix with 25-200 ng/ml pro-angiogenic growth factors (GF)...
May 10, 2018: Eye
https://www.readbyqxmd.com/read/29741098/monophosphoryl-lipid-a-prevents-impairment-of-medullary-thick-ascending-limb-hco-3-absorption-and-improves-plasma-hco-3-concentration-in-septic-mice
#2
Bruns A Watts, Thampi George, Edward R Sherwood, David W Good
Metabolic acidosis is the most common acid-base disorder in septic patients and is associated with increased mortality. Previously we demonstrated that sepsis induced by cecal ligation and puncture (CLP) impairs HCO3 - absorption in the MTAL by: 1) decreasing the intrinsic HCO3 - absorptive capacity, and 2) enhancing inhibition of HCO3 - absorption by LPS through upregulation of TLR4 signaling. Both effects depend on ERK activation. Monophosphoryl lipid A (MPLA) is a detoxified TLR4 agonist that enhances innate antimicrobial immunity and improves survival following sepsis...
May 9, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29734148/new-insights-into-cystic-kidney-diseases
#3
Toshio Mochizuki, Shiho Makabe, Yumi Aoyama, Hiroshi Kataoka, Kosaku Nitta
Hereditary cystic kidney diseases are considered as "ciliopathies" caused by abnormalities of the "primary cilia" situated on the tubules. As a result of dysplasia and dysfunction of cilia, formation of cysts occurs at various stages of life. Although occurring at a low incidence, hereditary cystic kidney diseases that develop from the fetal stage to childhood are diverse and are often associated with systemic disorders. The incidence of autosomal dominant polycystic kidney disease, which is the only adult-onset hereditary cystic kidney disease, is the highest among hereditary renal disorders...
2018: Contributions to Nephrology
https://www.readbyqxmd.com/read/29701177/a-case-of-infection-related-glomerulonephritis-with-massive-eosinophilic-infiltration
#4
Masahiro Okabe, Tsuyoshi Takamura, Akiko Tajiri, Nobuo Tsuboi, Masahiro Ishikawa, Makoto Ogura, Ryuji Ohashi, Takashi Oda, Takashi Yokoo
Infection-related glomerulonephritis (IRGN) is rarely complicated with eosinophil infiltration into the glomerulus. Here we report a case of eosinophilic proliferative glomerulonephritis related with infection. A 70-year-old man with respiratory symptoms displayed hypereosinophilia, hypocomplementemia, impaired renal function, and nephrotic syndrome. Renal biopsy revealed endocapillary proliferative glomerulonephritis with immunostaining for immunoglobulin G and complement 3, and subepithelial hump-like electron-dense deposits, thus fulfilling the criteria for IRGN...
April 27, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29689222/snare-complex-assembly-and-disassembly
#5
Tae-Young Yoon, Mary Munson
A fundamental hallmark of eukaryotic cells is their compartmentalization into functionally distinct organelles, including those of the secretory and endocytic pathways. Transport of cargo between these compartments and to/from the cell surface is mediated by membrane-bound vesicles and tubules. Delivery of cargo is facilitated by SNARE (soluble N-ethylmaleimide-sensitive factor attachment protein receptor)-mediated membrane fusion of vesicles with their target compartments. Vesicles contain a variety of cargos, including lipids, membrane proteins, signaling molecules, biosynthetic and hydrolytic enzymes, and the trafficking machinery itself...
April 23, 2018: Current Biology: CB
https://www.readbyqxmd.com/read/29686978/novel-aspects-of-renal-magnesium-homeostasis
#6
REVIEW
Paula Giménez-Mascarell, Carlotta Else Schirrmacher, Luis Alfonso Martínez-Cruz, Dominik Müller
Magnesium (Mg2+ ) is indispensable for several vital functions, such as neurotransmission, cardiac conductance, blood glucose, blood pressure regulation, and proper function of more than 300 enzymes. Thus, Mg2+ homeostasis is subject to tight regulation. Besides the fast and immediate regulation of plasma Mg2+ , a major part of Mg2+ homeostasis is realized by a concerted action of epithelial molecular structures that tightly control intestinal uptake and renal absorption. This mechanism is provided by a combination of para- and transcellular pathways...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29665107/a-case-of-a-rare-variant-of-klinefelter-syndrome-47-xy-i-x-q10
#7
T Kondo, S Kuroda, K Usui, K Mori, T Asai, T Takeshima, T Kawahara, H Hamanoue, H Uemura, Y Yumura
Klinefelter syndrome is a condition in which a male patient has one Y chromosome and one or more extra X chromosomes. It is the most common sex chromosome disorder. Klinefelter syndrome is distinguished by many clinical features, such as infertility, high gonadotropin and low testosterone levels, increased height, and sparse body and facial hair. We report the case of a 32-year-old man who visited our hospital complaining of male infertility. Semen analysis showed azoospermia, and chromosomal analysis revealed a 47,XY,i(X)(q10) karyotype, which is a rare variant of Klinefelter syndrome...
April 17, 2018: Andrologia
https://www.readbyqxmd.com/read/29627324/from-excitation-to-intracellular-ca-2-movements-in-skeletal-muscle-basic-aspects-and-related-clinical-disorders
#8
REVIEW
Bruno Allard
In skeletal muscle fiber, excitation-contraction coupling corresponds to the sequence of events occurring from action potential firing to initiation of contraction by an increase in cytosolic Ca2+ . These events are elicited in response to excitation of the motor neuron which induces trains of action potentials in the muscle cell that spread along the sarcolemma and in depth along the T-tubule membrane. Depolarization of the T-tubule membrane induces a conformational change in a protein complex, called the dihydropyridine receptor, which opens a calcium channel anchored in the membrane of the sarcoplasmic reticulum, called the ryanodine receptor, in charge of release of Ca2+ ions that activate contractile proteins...
March 9, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29626467/cooperation-of-rho-family-proteins-rac1-and-cdc42-in-cartilage-development-and-calcified-tissue-formation
#9
Mikiko Ikehata, Atsushi Yamada, Koji Fujita, Yuko Yoshida, Tadashi Kato, Akiko Sakashita, Hiroaki Ogata, Takehiko Iijima, Masahiko Kuroda, Daichi Chikazu, Ryutaro Kamijo
Rac1 and Cdc42, Rho family low molecular weight G proteins, are intracellular signaling factors that transmit various information from outside to inside cells. Primarily, they are known to control various biological activities mediated by actin cytoskeleton reorganization, such as cell proliferation, differentiation, and apoptosis. In order to investigate the functions of Rac1 and Cdc42 in bone formation, we prepared cartilage-specific double conditional knockout mice, Rac1fl/fl ; Cdc42fl/fl ; Col2-Cre (Rac1: Cdc42 dcKO mice), which died just after birth, similar to Cdc42fl/fl ; Col2-Cre mice (Cdc42 cKO mice)...
April 4, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29625388/crocin-reverses-unilateral-renal-ischemia-reperfusion-injury-induced-augmentation-of-oxidative-stress-and-toll-like-receptor-4-activity
#10
Hadeer O Abou-Hany, Hoda Atef, Eman Said, Hassan A Elkashef, Hatem A Salem
Renal Ischemia (RI) usually develops as a secondary manifestation of hypertension, various cardiovascular disorders and renal transplantation. It exerts hypoxic oxidative stress to kidneys, together with stimulation of several immune-mediated inflammatory cascades. Such events eventually damage renal tubules and glomeruli, driving acute kidney injury (AKI) and ultimately, renal failure. Crocin; the main bioactive constituent of Crocus sativus extract has been reported to demonstrate numerous pharmacological merits...
March 27, 2018: Environmental Toxicology and Pharmacology
https://www.readbyqxmd.com/read/29581513/the-cplane-protein-intu-protects-kidneys-from-ischemia-reperfusion-injury-by-targeting-stat1-for-degradation
#11
Shixuan Wang, Aimin Liu, Guangyu Wu, Han-Fei Ding, Shuang Huang, Stanley Nahman, Zheng Dong
Intu is known as a ciliogenesis and planar polarity effector (CPLANE) protein. Although roles for Intu have been reported during embryonic development and in the context of developmental disorders, its function and regulation in adult tissues remain poorly understood. Here we show that ablation of Intu specifically in kidney proximal tubules aggravates renal ischemia-reperfusion injury, and leads to defective post-injury ciliogenesis. We identify signal transducer and activator of transcription 1 (STAT1) as a novel interacting partner of Intu...
March 26, 2018: Nature Communications
https://www.readbyqxmd.com/read/29563333/osteopontin-deficiency-ameliorates-alport-pathology-by-preventing-tubular-metabolic-deficits
#12
Wen Ding, Keyvan Yousefi, Stefania Goncalves, Bradley J Goldstein, Alfonso L Sabater, Amy Kloosterboer, Portia Ritter, Guerline Lambert, Armando J Mendez, Lina A Shehadeh
Alport syndrome is a rare hereditary renal disorder with no etiologic therapy. We found that osteopontin (OPN) is highly expressed in the renal tubules of the Alport mouse and plays a causative pathological role. OPN genetic deletion ameliorated albuminuria, hypertension, tubulointerstitial proliferation, renal apoptosis, and hearing and visual deficits in the Alport mouse. In Alport renal tubules we found extensive cholesterol accumulation and increased protein expression of dynamin-3 (DNM3) and LDL receptor (LDLR) in addition to dysmorphic mitochondria with defective bioenergetics...
March 22, 2018: JCI Insight
https://www.readbyqxmd.com/read/29556787/dysregulation-of-the-renin-angiotensin-system-and-the-vasopressinergic-system-interactions-in-cardiovascular-disorders
#13
REVIEW
Ewa Szczepanska-Sadowska, Katarzyna Czarzasta, Agnieszka Cudnoch-Jedrzejewska
PURPOSE OF REVIEW: In many instances, the renin-angiotensin system (RAS) and the vasopressinergic system (VPS) are jointly activated by the same stimuli and engaged in the regulation of the same processes. RECENT FINDINGS: Angiotensin II (Ang II) and arginine vasopressin (AVP), which are the main active compounds of the RAS and the VPS, interact at several levels. Firstly, Ang II, acting on AT1 receptors (AT1R), plays a significant role in the release of AVP from vasopressinergic neurons and AVP, stimulating V1a receptors (V1aR), regulates the release of renin in the kidney...
March 19, 2018: Current Hypertension Reports
https://www.readbyqxmd.com/read/29553832/deficient-transient-receptor-potential-vanilloid-type-4-function-contributes-to-compromised-ca-2-i-homeostasis-in-human-autosomal-dominant-polycystic-kidney-disease-cells
#14
Viktor Tomilin, Gail A Reif, Oleg Zaika, Darren P Wallace, Oleh Pochynyuk
Autosomal-dominant polycystic kidney disease (ADPKD) is a devastating disorder that is characterized by a progressive decline in renal function as a result of the development of fluid-filled cysts. Defective flow-mediated [Ca2+ ]i responses and disrupted [Ca2+ ]i homeostasis have been repeatedly associated with cyst progression in ADPKD. We have previously demonstrated that the transient receptor potential vanilloid type 4 (TRPV4) channel is imperative for flow-mediated [Ca2+ ]i responses in murine distal renal tubule cells...
March 19, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29548867/ovotesticular-disorder-of-sex-development-presenting-as-an-acute-scrotum
#15
Seyed Ali Mirshahvalad, Paria Alinia, Pooya Hekmati, Nahid Nafisi, Abdol-Mohammad Kajbafzadeh
Hermaphroditism is known as ovotesticular disorder of sex development. A 14-year-old boy was admitted with right acute scrotum. Exploration revealed tunica rupture and hematoma, with no viable tissue. After one month, he was admitted again with left hemiscrotal pain. Microscopic examination of the left gonad demonstrated foci of hemorrhagic cysts, primordial follicles, and regions of seminiferous tubules. We preserved testicular tissue and the ovarian part was extracted completely. Long-term follow-up with his hormonal profile are reported...
March 13, 2018: Urology
https://www.readbyqxmd.com/read/29535000/pentoxifylline-inhibits-angiogenesis-via-decreasing-dll4-and-notch1-expression-in-mouse-proepicardial-explant-cultures
#16
Justyna Niderla-Bielińska, Krzysztof Bartkowiak, Bogdan Ciszek, Eric Czajkowski, Ewa Jankowska-Steifer, Alicja Krejner, Anna Ratajska
Pentoxifylline (PTX), a non-specific inhibitor of cAMP phosphodiesterases, is commonly used for treatment of peripheral vascular disorders although its direct action on endothelial cells is not well described. The aim of this study was to determine the influence of PTX on tubule formation and mRNA expression for angiogenesis-related proteins in endothelial cell line C166 and mouse proepicardial explants cultured on collagen. C166 cells and explants were stimulated with proangiogenic cocktail containing bFGF/VEGF-A120 /VEGF-A164 and with proangiogenic cocktail enriched with PTX...
May 15, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29514215/loss-of-cln7-results-in-depletion-of-soluble-lysosomal-proteins-and-impaired-mtor-reactivation
#17
Tatyana Danyukova, Khandsuren Ariunbat, Melanie Thelen, Nahal Brocke-Ahmadinejad, Sara E Mole, Stephan Storch
Defects in the MFSD8 gene encoding the lysosomal membrane protein CLN7 lead to CLN7 disease, a neurodegenerative lysosomal storage disorder belonging to the group of neuronal ceroid lipofuscinoses. Here, we have performed a SILAC-based quantitative analysis of the lysosomal proteome using Cln7-deficient mouse embryonic fibroblasts (MEFs) from a Cln7 knockout (ko) mouse model. From 3335 different proteins identified, we detected 56 soluble lysosomal proteins and 29 highly abundant lysosomal membrane proteins...
May 15, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29509141/-fanconi-bickel-syndrom-a-novel-genetic-disease-in-original-braunvieh
#18
S Joller, M Stettler, I Locher, M Dettwiler, F Seefried, M Meylan, C Drögemüller
This case report describes a new genetic disease of the Braunvieh breed in Switzerland. The bovine disorder also occurs in German Fleckvieh, and corresponds to human Fanconi-Bickel syndrome which is an inherited glycogen storage disease caused by mutations of the SLC2A2 gene encoding the glucose transporter GLUT2. This case report describes a single affected Original Braunvieh calf genotyped as homozygous for the FH2-associated SLC2A2 frame shift mutation. The clinical examination showed stunted growth, polyuria and polydipsia, as well as poor claw horn and coat quality...
March 2018: Schweizer Archiv Für Tierheilkunde
https://www.readbyqxmd.com/read/29504143/a-case-report-of-ovotesticular-disorder-of-sex-development-ot-dsd-in-a-baboon-papio-spp-and-a-brief-review-of-the-non-human-primate-literature
#19
REVIEW
Ekaterina Perminov, Sara Mangosing, Alexandra Confer, Olga Gonzalez, Jason R Crawford, Natalia Schlabritz-Loutsevitch, Shyamesh Kumar, Edward Dick
Disorders of sexual development are rare in non-human primates. We report a case of true hermaphroditism in a 19-year-old, nulliparous, female baboon (Papio spp.). At necropsy, the animal was obese with adequate muscle mass and hydration. Reproductive organs appeared normal with the exception of 2 firm nodular structures in the myometrium (1-1.5 cm diameter) and a thickened, dark endocervical mucosa. Histologically, both gonads were ovotestes and contained discrete areas of ovarian and testicular tissue. There were follicles in various stages of development surrounded by ovarian stroma...
June 2018: Journal of Medical Primatology
https://www.readbyqxmd.com/read/29490976/proximal-tubular-secretory-clearance-a-neglected-partner-of-kidney-function
#20
REVIEW
Ke Wang, Bryan Kestenbaum
The secretion of small molecules by the proximal tubules of the kidneys represents a vital homeostatic function for rapidly clearing endogenous solutes and medications from the circulation. After filtration at the glomerulus, renal blood flow is directed through a network of peritubular capillaries, where transporters of the proximal tubules actively secrete putative uremic toxins and hundreds of commonly prescribed drugs into the urine, including protein-bound substances that cannot readily cross the glomerular basement membrane...
February 28, 2018: Clinical Journal of the American Society of Nephrology: CJASN
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