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K V Firsov, A S Kotov
Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system. Manifestations of Fabry disease include progressive renal and cardiac insufficiency, neuropathic pain, stroke and cerebral disease, skin and gastrointestinal symptoms...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Zhi-Yuan Li, Zhao-Li Chen, Ting Zhang, Chao Wei, Wei-Yun Shi
The corneal epithelium plays important roles in the maintenance of corneal transparency for good vision, and acts as a protective barrier against foreign insults. Structural and functional changes with aging in the corneal epithelium have been documented. Here we found that transforming growth factor-β (TGF-β) is highly expressed in the elderly donor corneal epithelium, as are senescence-associated genes, such as p16 and p21. In human corneal epithelial cell (HCEC) models, TGF-β induces cellular senescence, characterized by increased SA-β-gal positive cells and elevated expression of p16 and p21...
October 6, 2016: Aging
Thorsten M Seyler, Daniel N Bracey, Johannes F Plate, Mark O Lively, Sandeep Mannava, Thomas L Smith, Justin M Saul, Gary G Poehling, Mark E Van Dyke, Patrick W Whitlock
PURPOSE: To evaluate the biological, immunological, and biomechanical properties of a scaffold derived by architectural modification of a fresh-frozen porcine patella tendon using a decellularization protocol that combines physical, chemical, and enzymatic modalities. METHODS: Porcine patellar tendons were processed using a decellularization and oxidation protocol that combines physical, chemical, and enzymatic modalities. Scaffolds (n = 88) were compared with native tendons (n = 70) using histologic, structural (scanning electron microscopy, porosimetry, and tensile testing), biochemical (mass spectrometry, peracetic acid reduction, DNA quantification, alpha-galactosidase [α-gal] content), as well as in vitro immunologic (cytocompatibility, cytokine induction) and in vivo immunologic nonhuman primate analyses...
September 27, 2016: Arthroscopy: the Journal of Arthroscopic & related Surgery
Ming-Wei Liu, Rong Liu, Hai-Yin Wu, Wei Zhang, Jing Xia, Min-Na Dong, Wen Yu, Qiang Wang, Feng-Mei Xie, Rui Wang, Yun-Qiao Huang, Chuan-Yun Qian
Xuebijing injection (XBJ) has long been used to treat infectious diseases in China. The therapeutic effect of XBJ is probably associated with anti-inflammatory effects. However, the precise mechanisms responsible for the effects of XBJ remain unknown. The present study was conducted in order to evaluate the protective effects of XBJ in a rat model of D-galactosamine (D-Gal)- and lipopolysaccharide (LPS)‑induced acute liver injury. In the present study, the rats were injected with D-Gal and LPS intraperitoneally to induce acute liver injury...
September 23, 2016: International Journal of Molecular Medicine
J Chen, B J Liu, C Du, Q Cao, M Li, H Feng
OBJECTIVE: To explore the effect of microRNA-203(miR-203) on the expression of collagen type IV alpha 4(COL4A4) and its role in oral submucous fibrosis(OSF). METHODS: It has been revealed that COL4A4 is the putative target of miR-203 by retrieving a variety of bioinformatics software tools. The expression of COL4A4 protein in the indicated tissues was examined by Western blotting analysis and the potential binding sites of miR-203 and COL4A4-3'UTR was analyzed by using bioinformatics...
September 2016: Zhonghua Kou Qiang Yi Xue za Zhi, Zhonghua Kouqiang Yixue Zazhi, Chinese Journal of Stomatology
Cassiano Augusto Braga Silva, Fellype Carvalho Barreto, Marlene Antonia Dos Reis, José Andrade Moura Junior, Constança Margarida Sampaio Cruz
INTRODUCTION: Fabry disease (FD) is a lysosomal storage disorder caused by enzyme α galactosidase A (α-Gal A) deficiency due to mutations in the galactosidase alpha (GLA) gene. It leads to damage several organs, such as the kidneys, due to progressive accumulation of glycosphingolipids. OBJECTIVE: To estimate the prevalence of FD among male hemodialysis (HD) patients in a northern state of Brazil. METHODS: Screening was performed using a dried blood spot on filter paper to identify patients with low α-Gal A enzyme activity (≤2...
August 31, 2016: Nephron
Tomohiro Arikawa, Shengjun Liao, Hiroki Shimada, Tomoki Inoue, Hiromi Sakata-Haga, Takanori Nakamura, Toshihisa Hatta, Hiroki Shoji
Placental development and trophoblast invasion of the maternal endometrium establish the maternal-fetal interface, which is critical for the developing embryo and fetus. Herein we show that overexpression of Galectin-4 (Gal-4) during trophoblast differentiation inhibited the enlargement of Rcho-1 cells (a model for rat trophoblast differentiation) and promoted cell-cell adhesion, whereas trophoblast specific markers and MMP-9 activity were not affected. In the rat placenta, microtubule associated protein 1 light chain 3 alpha (LC3) protein, an autophagy marker, is highly expressed on the maternal side of the decidua where Gal-4 expression is weak...
2016: Scientific Reports
Jinfeng Liu, Shiguang Huang, Xin-Zhuan Su, Jianping Song, Fangli Lu
Malaria-associated acute lung injury (ALI) is a frequent complication of severe malaria that is often caused by "excessive" immune responses. To better understand the mechanism of ALI in malaria infection, here we investigated the roles of galectin (Gal)-1, 3, 8, 9 and the receptors of Gal-9 (Tim-3, CD44, CD137, and PDI) in malaria-induced ALI. We injected alpha (α)-lactose into mice-infected with Plasmodium berghei ANKA (PbANKA) to block galectins and found significantly elevated total proteins in bronchoalveolar lavage fluid, higher parasitemia and tissue parasite burden, and increased numbers of CD68(+) alveolar macrophages as well as apoptotic cells in the lungs after blockage...
2016: Scientific Reports
Scott P Commins
The syndrome of delayed allergic reactions to the carbohydrate galactose-alpha-1,3-galactose ("alpha-gal") has become increasingly recognized in allergy and immunology clinics regionally throughout the southeastern USA. Due to the increasing awareness of this unique food allergy, cases have been identified in the northeastern and central USA as well as in Central and South America, Europe, Asia, Scandinavia, and Australia. Clinically, alpha-gal allergy is characterized by reactions to non-primate mammalian meat (e...
September 2016: Current Allergy and Asthma Reports
Gergely Kacso, Dora Ravasz, Judit Doczi, Beáta Németh, Ory Madgar, Ann Saada, Polina Ilin, Chaya Miller, Elsebet Ostergaard, Iordan Iordanov, Daniel Adams, Zsuzsanna Vargedo, Masatake Araki, Kimi Araki, Mai Nakahara, Haruka Ito, Aniko Gál, Mária J Molnár, Zsolt Nagy, Attila Patocs, Vera Adam-Vizi, Christos Chinopoulos
Succinate-CoA ligase (SUCL) is a heterodimer enzyme composed of Suclg1 α-subunit and a substrate-specific Sucla2 or Suclg2 β-subunit yielding ATP or GTP, respectively. In humans, the deficiency of this enzyme leads to encephalomyopathy with or without methylmalonyl aciduria, in addition to resulting in mitochondrial DNA depletion. We generated mice lacking either one Sucla2 or Suclg2 allele. Sucla2 heterozygote mice exhibited tissue- and age-dependent decreases in Sucla2 expression associated with decreases in ATP-forming activity, but rebound increases in cardiac Suclg2 expression and GTP-forming activity...
October 15, 2016: Biochemical Journal
Wei-Chieh Cheng, Jen-Hon Wang, Huang-Yi Li, Sheng-Jhih Lu, Jia-Ming Hu, Wen-Yi Yun, Cheng-Hsin Chiu, Wen-Bin Yang, Yin-Hsiu Chien, Wuh-Liang Hwu
A unique molecular library consisting of all sixteen synthetic ADMDP (1-aminodeoxy-DMDP) stereoisomers has been prepared and evaluated for inhibitory activity against α-Gal A, and ability to impart thermal stabilization of this enzyme. The results of this testing led us to develop a novel pharmacological chaperone for the treatment of Fabry disease. 3-Epimer ADMDP was found to be an effective pharmacological chaperone, able to rescue α-Gal A activity in the lymphoblast of the N215S Fabry patient-derived cell line, without impairment of cellular β-galactosidase activity...
November 10, 2016: European Journal of Medicinal Chemistry
Isidro Machado, María Victoria López-Soto, Albadio Samir Pérez-López, Carlos Domínguez-Álvarez, Antonio Llombart-Bosch
Hyaline globules and papillary fragments in cytologic samples from two intra-abdominal tumors in young females are presented including the cytological features and the correlation with the histopathologic and immunohistochemical findings. In the first case a cytologic study from an ovarian mass showed papillary structures and isolated tumor cells with epithelioid morphology, irregular reniform-like nuclear contour, pale or vacuolated cytoplasm, abundant hyaline globules and occasional glomeruloid structures resembling Schiller-Duval bodies...
July 13, 2016: Diagnostic Cytopathology
Bishu Ganguly, Balaji Balasa, Lyubov Efros, Paul R Hinton, Stephen Hartman, Archana Thakur, Joanna M Xiong, Brian Schmidt, Randy R Robinson, Thierry Sornasse, Vladimir Vexler, James P Sheridan
The CD25-binding antibody daclizumab high-yield process (DAC HYP) is an interleukin (IL)-2 signal modulating antibody that shares primary amino acid sequence and CD25 binding affinity with Zenapax®, a distinct form of daclizumab, which was approved for the prevention of acute organ rejection in patients receiving renal transplants as part of an immunosuppressive regimen that includes cyclosporine and corticosteroids. Comparison of the physicochemical properties of the two antibody forms revealed the glycosylation profile of DAC HYP differs from Zenapax in both glycan distribution and the types of oligosaccharides, most notably high-mannose, galactosylated and galactose-alpha-1,3-galactose (alpha-Gal) oligosaccharides, resulting in a DAC HYP antibody material that is structurally distinct from Zenapax...
July 1, 2016: MAbs
E P Smorodin, B L Sergeyev
BACKGROUND: The serum levels of IgG antibodies reactive to glycoconjugates (TF, Tn and αGal) were found to be associated with prognosis of gastrointestinal cancer patients. AIM: To study the relation between the levels of serum antibodies to TF-pAA, Tn-PAA and αGal-PAA polyacrylamide-based glycoconjugates and survival in breast cancer. MATERIALS AND METHODS: The preoperative level of IgG antibodies was analysed in the serum of patients (n = 59) using ELISA with polyacrylamide-glycoconjugates namely, TF-pAA (amide-type), and ethanolamide-conjugates Tn-PAA and αGal-PAA...
June 2016: Experimental Oncology
Natália Miki-Rosário, Reynaldo Jesus Garcia Filho, Jairo Greco Garcia, Gal Moreira Dini, Andrew Bottomley, Edward Chow, Miguel Sabino Neto
BACKGROUND: The aim of the present study was to conduct a cross-cultural adaptation (with translation into Brazilian Portuguese) and validation of the European Organization for Research and Treatment of Cancer-Quality of Life Questionnaire-Bone Metastases-22 (EORTC QLQ-BM22). METHODS: Ninety-five bone metastasis patients (31 men and 64 women, mean age 58.36±8.90 years) took part in the investigation. The translation guide of the EORTC was used to translate from English into Brazilian Portuguese and adapt the instrument culturally...
July 2016: Annals of Palliative Medicine
Joseph Platz, Nicholas R Bonenfant, Franziska E Uhl, Amy L Coffey, Tristan McKnight, Charles Parsons, Dino Sokocevic, Zachary D Borg, Ying-Wai Lam, Bin Deng, Julia G Fields, Michael DeSarno, Roberto Loi, Andrew M Hoffman, John Bianchi, Brian Dacken, Thomas Petersen, Darcy E Wagner, Daniel J Weiss
BACKGROUND: A novel potential approach for lung transplantation could be to utilize xenogeneic decellularized pig lung scaffolds that are recellularized with human lung cells. However, pig tissues express several immunogenic proteins, notably galactosylated cell surface glycoproteins resulting from alpha 1,3 galactosyltransferase (α-gal) activity, that could conceivably prevent effective use. Use of lungs from α-gal knock out (α-gal KO) pigs presents a potential alternative and thus comparative de- and recellularization of wild-type and α-gal KO pig lungs was assessed...
August 2016: Tissue Engineering. Part C, Methods
Thomas A E Platts-Mills, Alexander J Schuyler, Elizabeth A Erwin, Scott P Commins, Judith A Woodfolk
Traditionally, the concept of allergy implied an abnormal response to an otherwise benign agent (eg, pollen or food), with an easily identifiable relationship between exposure and disease. However, there are syndromes in which the relationship between exposure to the relevant allergen and the "allergic" disease is not clear. In these cases the presence of specific IgE antibodies can play an important role in identifying the relevant allergen and provide a guide to therapy. Good examples include chronic asthma and exposure to perennial indoor allergens and asthma related to fungal infection...
June 2016: Journal of Allergy and Clinical Immunology
Zhao Liu, Long Wang, Junhua Yang, Abhik Bandyopadhyay, Virginia Kaklamani, Shui Wang, Lu-Zhe Sun
Exposure to estrogen has long been associated with an increased risk of developing breast cancer. However, how estrogen signaling promotes breast carcinogenesis remains elusive. Senescence is known as an important protective response to oncogenic events. We aimed to elucidate the role of estrogen receptor alpha (ERα) on senescence in transformed human mammary epithelial cells and breast cancer cells. Our results show that ectopic expression of oncoprotein H-ras-V12 in immortalized human mammary epithelial cells (HMEC) significantly inhibited the phosphorylation of the retinoblastoma protein (Rb) and increased the activity of the senescence-associated beta-galactosidase (SA-β-Gal)...
June 1, 2016: Oncotarget
Ling Li, Xiaoguang Wang, Xinhua Zhang, Mei Guo, Tieling Liu
BACKGROUND: The RIN transcription factor is one of the MADS box family members and predominantly controls fruit ripening. In this study, effort was made to demonstrate the regulation network of RIN transcription factor during tomato fruit ripening and softening. Novel RIN target genes were identified by proteomics, electrophoresis mobility shift assay (EMSA) and chromatin immunoprecipitation (ChIP) analysis. RESULTS: Over 700 protein spots were achieved by two-dimensional electrophoresis (2-DE) and forty-two of them were finally identified...
June 1, 2016: Journal of the Science of Food and Agriculture
Robert B Hawkins, Helena L Frischtak, Irving L Kron, Ravi K Ghanta
We present the cases of two patients with bioprosthetic aortic valves who developed an allergy to alpha-gal. Each had premature degeneration of their bioprosthesis and demonstrated rapidly increasing transvalvular gradients after development of their allergy. Each underwent successful replacement with a mechanical aortic valve within 1-2 years of symptom onset. doi: 10.1111/jocs.12764 (J Card Surg 2016;31:446-448).
July 2016: Journal of Cardiac Surgery
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