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https://www.readbyqxmd.com/read/28430766/linear-immunoglobulin-a-dermatosis-a-case-report
#1
Fernando Valenzuela Ahumada, Roberto Bustos Macaya, Gabriela Paz Romero Morgado, Margarita Sánchez Chacón
We present the case of a sixty five year old woman with two months history of pruritus and hyperpigmented annular lesions on the trunk, buttocks and upper extremities. In addition, she presents vesicles with healthy skin on the basis, in the flexor aspect of wrists. No evidence of mucosal involvement. Histological study showed subepidermal vesicular dermatitis with inflammatory infiltrate of neutrophils and eosinophils. Direct immunofluorescence evidenced linear and continuous deposition of immunoglobulin A in basement membrane zone, compatible with linear immunoglobulin A disease...
April 4, 2017: Medwave
https://www.readbyqxmd.com/read/28428258/deletion-of-inositol-requiring-enzyme-1%C3%AE-in-podocytes-disrupts-glomerular-capillary-integrity-and-autophagy
#2
Daniel Robert Kaufman, Joan Papillon, Louise Larose, Takao Iwawaki, Andrey V Cybulsky
Inositol-requiring enzyme-1α (IRE1α) is an endoplasmic reticulum (ER)-transmembrane endoribonuclease-kinase, which plays an essential function in extraembryonic tissues during normal development, and is activated during ER stress. To address the functional role of IRE1α in glomerular podocytes, we produced podocyte-specific IRE1α deletion mice. In male mice, deletion of IRE1α in podocytes resulted in albuminuria beginning at 5 months of age, and worsening with time. Electron microscopy revealed focal podocyte foot process effacement in 9-month old male IRE1α deletion mice, as well as microvillous transformation of podocyte plasma membranes...
April 20, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28428179/adamts13-controls-vascular-remodeling-by-modifying-vwf-reactivity-during-stroke-recovery
#3
Haochen Xu, Yongliang Cao, Xing Yang, Ping Cai, Lijing Kang, Ximin Zhu, Haiyu Luo, Lu Lu, Lixiang Wei, Xiaofei Bai, Yuanbo Zhu, Bing-Qiao Zhao, Wenying Fan
Angiogenic response is essential for ischemic brain repair. However, the mechanisms leading to postischemic neovascularization have not been established, and no therapies have been identified. The VWF-cleaving protease ADAMTS13 is required for endothelial tube formation in vitro However, there is currently no in vivo evidence supporting a function of ADAMTS13 in angiogenesis. Here we show that mice deficient in ADAMTS13 exhibited significantly reduced neovascularization, brain capillary perfusion, pericyte and smooth muscle cell coverage on microvessels, expression of the tight junction and basement membrane proteins, and accelerated blood-brain barrier (BBB) breakdown and extravascular deposits of serum proteins in the peri-infarct cortex at 14 days after stroke...
April 20, 2017: Blood
https://www.readbyqxmd.com/read/28427981/ultrastructure-of-pericystic-or-intracystic-blood-vessels-in-epidermoid-cysts-a-transmission-electron-microscopy-study-laboratory-investigation
#4
Xiao-Hui Ren, Jun Ma, Chun Zeng, Yi-Lin Sun, Song Lin
OBJECTIVES: Recently, we reported the tendency towards the spontaneous hemorrhage in both pre- and post-operative periods of patients with intracranial epidermoid cyst (EC). According to our experience this tendency of spontaneous hemorrhage was partly due to the pathologic blood vessels adjacent to the EC. This article was designed to testify this hypothesis. MATERIALS & METHODS: 23 removable pericystic or intracystic blood vessels from 17 EC patients were collected during surgery, and then were examined by transmission electron microscope...
April 17, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28424209/the-sulfilimine-cross-link-of-collagen-iv-contributes-to-kidney-tubular-basement-membrane-stiffness
#5
Gautam Bhave, Selene Colon, Nicholas Ferrell
Basement membranes (BMs) are a specialized form of extracellular matrix (ECM) which underlie nearly all cell layers and provide structural support for tissues and interact with cell surface receptors to determine cell behavior. Both macromolecular composition and stiffness of BM influence cell-BM interactions. Collagen IV is major constituent of BM that forms an extensively cross-linked oligomeric network. Its deficiency leads to BM mechanical instability as observed with glomerular basement membrane (GBM) in Alport Syndrome...
April 19, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28420996/chronic-water-pipe-smoke-exposure-induces-injurious-effects-to-reproductive-system-in-male-mice
#6
Badreldin H Ali, Khalid A Al Balushi, Mohammed Ashique, Asem Shalaby, Mohammed A Al Kindi, Sirin A Adham, Turan Karaca, Sumaya Beegam, Priya Yuvaraju, Abderrahim Nemmar
There is a global increase in the popularity of water-pipe tobacco smoking including in Europe and North America. Nevertheless, little is known about the male reproductive effects of water-pipe smoke (WPS), especially after long-term exposure. Here, we assessed effects of WPS exposure (30 min/day) in male mice for 6 months. Control mice were exposed to air-only for the same period of time. Twenty-four hours after the last exposure, testicular histopathology, and markers of inflammation and oxidative stress, and the tyrosine-protein kinase vascular endothelial growth factor receptor 1 (VEGFR1) were assessed in testicular homogenates...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28419360/p4ha1-mutations-cause-a-unique-congenital-disorder-of-connective-tissue-involving-tendon-bone-muscle-and-the-eye
#7
Yaqun Zou, Sandra Donkervoort, Antti M Salo, A Reghan Foley, Aileen M Barnes, Ying Hu, Elena Makareeva, Meganne E Leach, Payam Mohassel, Jahannaz Dastgir, Matthew A Deardorff, Ronald D Cohn, Wendy O DiNonno, Fransiska Malfait, Monkol Lek, Sergey Leikin, Joan C Marini, Johanna Myllyharju, Carsten G Bönnemann
Collagen prolyl 4-hydroxylases (C-P4Hs) play a central role in the formation and stabilization of the triple helical domain of collagens. P4HA1 encodes the catalytic α(I) subunit of the main C-P4H isoenzyme (C-P4H-I). We now report human bi-allelic P4HA1 mutations in a family with a congenital-onset disorder of connective tissue, manifesting as early-onset joint hypermobility, joint contractures, muscle weakness and bone dysplasia as well as high myopia, with evidence of clinical improvement of motor function over time in the surviving patient...
April 13, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28419296/urinary-podocyte-and-tgf-%C3%AE-1-mrna-as-markers-for-disease-activity-and-progression-in-anti-glomerular-basement-membrane-nephritis
#8
Akihiro Fukuda, Akihiro Minakawa, Yuji Sato, Takashi Iwakiri, Shuji Iwatsubo, Hiroyuki Komatsu, Masao Kikuchi, Kazuo Kitamura, Roger C Wiggins, Shouichi Fujimoto
Background.: Podocyte depletion causes glomerulosclerosis, with persistent podocyte loss being a major factor driving disease progression. Urinary podocyte mRNA is potentially useful for monitoring disease progression in both animal models and in humans. To determine whether the same principles apply to crescentic glomerular injury, a rat model of anti-glomerular basement membrane (anti-GBM) nephritis was studied in parallel with a patient with anti-GBM nephritis. Methods...
April 17, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28418422/a-disease-model-of-diabetic-nephropathy-in-a-glomerulus-on-a-chip-microdevice
#9
Li Wang, Tingting Tao, Wentao Su, Hao Yu, Yue Yu, Jianhua Qin
Diabetic nephropathy is a major chronic renal complication of diabetes mellitus, and is the leading cause of end-stage kidney diseases. Establishing a disease model of diabetic nephropathy in vitro can accelerate the understanding of its mechanisms and pharmaceutical development. We provide the proof-of-principle for using a glomerulus-on-a-chip microdevice that reconstitutes organ-level kidney functions to create a human disease model of early stage diabetic nephropathy on chip. The microfluidic device, which recapitulates the glomerular microenvironment, consists of parallel channels lined by isolated primary glomerular microtissues that experience fluid flow to mimic the glomerular filtration barrier (GFB), including glomerular endothelial cells, 3D basement membrane and podocytes...
April 18, 2017: Lab on a Chip
https://www.readbyqxmd.com/read/28418331/collagen-iv-and-basement-membrane-at-the-evolutionary-dawn-of-metazoan-tissues
#10
Aaron L Fidler, Carl E Darris, Sergei V Chetyrkin, Vadim K Pedchenko, Sergei P Boudko, Kyle L Brown, W Gray Jerome, Julie K Hudson, Antonis Rokas, Billy G Hudson
The role of the cellular microenvironment in enabling metazoan tissue genesis remains obscure. Ctenophora has recently emerged as one of the earliest-branching extant animal phyla, providing a unique opportunity to explore the evolutionary role of the cellular microenvironment in tissue genesis. Here, we characterized the extracellular matrix (ECM), with a focus on collagen IV and its variant, spongin short-chain collagens, of non-bilaterian animal phyla. We identified basement membrane (BM) and collagen IV in Ctenophora, and show that the structural and genomic features of collagen IV are homologous to those of non-bilaterian animal phyla and Bilateria...
April 18, 2017: ELife
https://www.readbyqxmd.com/read/28415787/knockdown-of-p4ha1-inhibits-neovascularization-via-targeting-glioma-stem-cell-endothelial-cell-transdifferentiation-and-disrupting-vascular-basement-membrane
#11
Yiqiang Zhou, Guishan Jin, Ruifang Mi, Junwen Zhang, Jin Zhang, Hengzhou Xu, Sen Cheng, Yunsheng Zhang, Wenjie Song, Fusheng Liu
Emerging evidence has demonstrated transdifferentiation process of glioma stem cells (GSCs) into endothelial cells (ECs) in glioma neovascularization. Herein, we focused on screening for genes that were differentially expressed in the transdifferentiation process using microarray analysis. Bioinformatics analysis revealed differential expression of the prolyl 4-hydroxylase subunit alpha-1 (P4HA1) gene. We determined that P4HA1 expression was correlated with histological grade, the level of Ki67 and microvessel density (MVD) in human glioma specimens...
March 16, 2017: Oncotarget
https://www.readbyqxmd.com/read/28412362/probing-the-acidic-residue-within-the-integrin-binding-site-of-laminin-511-that-interacts-with-the-metal-ion-dependent-adhesion-site-of-%C3%AE-6%C3%AE-1-integrin
#12
Yukimasa Taniguchi, Shaoliang Li, Mamoru Takizawa, Eriko Oonishi, Junko Toga, Emiko Yagi, Kiyotoshi Sekiguchi
Laminins are major cell-adhesive proteins of basement membranes that interact with integrins in a divalent cation-dependent manner. Laminin-511 consists of α5, β1, and γ1 chains, of which three laminin globular domains of the α5 chain (α5/LG1-3) and a Glu residue in the C-terminal tail of chain γ1 (γ1-Glu1607) are required for binding to integrins. However, it remains unsettled whether the Glu residue in the γ1 tail is involved in integrin binding by coordinating the metal ion in the metal ion-dependent adhesion site of β1 integrin (β1-MIDAS), or by stabilizing the conformation of α5/LG1-3...
April 12, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28411477/comparative-surface-energetic-study-of-matrigel-%C3%A2-and-collagen-i-interactions-with-endothelial-cells
#13
Michael J Hill, Debanjan Sarkar
Understanding of the surface energetic aspects of the spontaneously deposited proteins on biomaterial surfaces and how this influences cell adhesion and differentiation is an area of regenerative medicine that has not received adequate attention. Current controversies surround the role of the biomaterial substratum surface chemistry, the range of influence of said substratum, and the effects of different surface energy components of the protein interface. Endothelial cells (ECs) are a highly important cell type for regenerative medicine applications, such as tissue engineering, and In-vivo they interact with collagen I based stromal tissue and basement membranes producing different behavioral outcomes...
April 4, 2017: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/28410377/plasma-from-patients-with-anti-glomerular-basement-membrane-disease-could-recognize-microbial-peptides
#14
Jian-Nan Li, Xiaoyu Jia, Yongqiang Wang, Can Xie, Taijiao Jiang, Zhao Cui, Ming-Hui Zhao
Infection has long been suspected as a trigger of autoimmune diseases, and molecular mimicry mechanism was hypothesized in this study. Microbe originated peptides were searched from the Uniprot database based on a previous defined critical amino acid motif within α3129-150, isoleucine137, tryptophan140, glycine142, phenylalanine 143 and phenylalanine 145. 23826 microbial peptides were identified using our searching strategy, among which seven were related with human infections. Circulating IgG and IgM antibodies against the seven microbial peptides were detected using ELISA in 76 patients with anti-GBM disease...
2017: PloS One
https://www.readbyqxmd.com/read/28408750/transcriptional-regulatory-networks-underlying-the-reprogramming-of-spermatogonial-stem-cells-to-multipotent-stem-cells
#15
Hoe-Su Jeong, Jinhyuk Bhin, Hyung Joon Kim, Daehee Hwang, Dong Ryul Lee, Kye-Seong Kim
Spermatogonial stem cells (SSCs) are germline stem cells located along the basement membrane of seminiferous tubules in testes. Recently, SSCs were shown to be reprogrammed into multipotent SSCs (mSSCs). However, both the key factors and biological networks underlying this reprogramming remain elusive. Here, we present transcriptional regulatory networks (TRNs) that control cellular processes related to the SSC-to-mSSC reprogramming. Previously, we established intermediate SSCs (iSSCs) undergoing the transition to mSSCs and generated gene expression profiles of SSCs, iSSCs and mSSCs...
April 14, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/28408317/involvement-of-puma-in-pericyte-migration-induced-by-methamphetamine
#16
Yanhong Zhang, Yuan Zhang, Ying Bai, Jie Chao, Gang Hu, Xufeng Chen, Honghong Yao
Mounting evidence indicates that methamphetamine causes blood-brain barrier damage, with emphasis on endothelial cells. The role of pericytes in methamphetamine-induced BBB damage remains unknown. Our study demonstrated that methamphetamine increased the migration of pericytes from the endothelial basement membrane. However, the detailed mechanisms underlying this process remain poorly understood. Thus, we examined the molecular mechanisms involved in methamphetamine-induced pericyte migration. The results showed that exposure of C3H/10T1/2 cells and HBVPs to methamphetamine increased PUMA expression via activation of the sigma-1 receptor, MAPK and Akt/PI3K pathways...
April 10, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28408249/temozolomide-does-not-influence-the-transcription-or-activity-of-matrix-metalloproteinases-9-and-2-in-glioma-cell-lines
#17
Yuta Suzuki, Kouki Fujioka, Keiichi Ikeda, Yuichi Murayama, Yoshinobu Manome
Glioblastoma multiforme (GBM) is a treatment-resistant malignancy with poor prognosis. Temozolomide (TMZ) is widely used as a first-line drug for GBM. Although this improves patient prognosis, it does not completely eradicate the tumour. Even after total surgical resection, GBM can exhibit uncontrollable invasiveness at the tumour margins owing to activation of matrix metalloproteinases (MMPs) such as MMP-2 and -9; these degrade collagen IV in the basement membrane, which normally prevents cancer invasion. TMZ induces DNA damage and activates transcription factors including c-jun, c-fos, nuclear factor-κβ, and early growth response protein-1, which have putative binding sites on the MMP-9 promoter...
April 10, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28407818/-clinical-and-pathological-features-and-the-misdiagnosis-of-childhood-alport-syndrome-a-retrospective-analysis-of-91-cases
#18
Yan-Zhen Chen, Liang-Zhong Sun, Hai-Yan Wang, Xiao-Yun Jiang, Ying Mo, Zhi-Hui Yue, Hua-Mu Chen, Ting Liu, Hong-Rong Lin
OBJECTIVE: To explore the clinical and pathological features and the diagnosis of childhood Alport syndrome (AS). METHODS: A retrospective analysis was performed on clinical data of 91 children with AS. RESULTS: Hematuria was observed in all 91 patients, of whom 86 were accompanied with proteinuria. Sixty-one children with X-Linked AS (XL-AS) had positive family history. Renal biopsy was performed on 82 children. Mild to moderate mesangial proliferation was observed in 74 cases...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28404521/diabetic-complications-in-the-cornea
#19
Alexander V Ljubimov
Diabetic corneal alterations, such as delayed epithelial wound healing, edema, recurrent erosions, neuropathy/loss of sensitivity, and tear film changes are frequent but underdiagnosed complications of both type 1 (insulin-dependent) and type 2 (non-insulin-dependent) diabetes mellitus. The disease affects corneal epithelium, corneal nerves, tear film, and to a lesser extent, endothelium, and also conjunctiva. These abnormalities may appear or become exacerbated following trauma, as well as various surgeries including retinal, cataract or refractive...
April 9, 2017: Vision Research
https://www.readbyqxmd.com/read/28403904/pre-existing-chronic-interstitial-pneumonia-is-a-poor-prognostic-factor-of-goodpasture-s-syndrome-a-case-report-and-review-of-the-literature
#20
Hiroki Tashiro, Koichiro Takahashi, Yuki Ikeda, Saori Uchiumi, Makoto Fukuda, Miyazono Motoaki, Shinya Kimura, Naoko Sueoka-Aragane
BACKGROUND: Goodpasture's syndrome is a rare disease that is characterized by rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. CASE PRESENTATION: A 71-year-old Japanese man who had chronic interstitial pneumonia was diagnosed as having Goodpasture's syndrome. Both anti-glomerular basement membrane antibody and myeloperoxidase anti-neutrophil cytoplasmic antibody were increased. Despite intensive treatments, including mechanical ventilation, he died from respiratory failure...
April 13, 2017: Journal of Medical Case Reports
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