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https://www.readbyqxmd.com/read/28515353/chaperone-mediated-autophagy-substrate-proteins-in-cancer
#1
REVIEW
Ying Tang, Xiong-Wen Wang, Zhan-Hua Liu, Yun-Ming Sun, Yu-Xin Tang, Dai-Han Zhou
All intracellular proteins undergo continuous synthesis and degradation. Chaperone-mediated autophagy (CMA) is necessary to maintain cellular homeostasis through turnover of cytosolic proteins (substrate proteins). This degradation involves a series of substrate proteins including both cancer promoters and suppressors. Since activating or inhibiting CMA pathway to treat cancer is still debated, targeting to the CMA substrate proteins provides a novel direction. We summarize the cancer-associated substrate proteins which are degraded by CMA...
May 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28508651/sugar-breathing-glycopolymersomes-for-regulating-glucose-level
#2
Yufen Xiao, Hui Sun, Jianzhong Du
Diabetes mellitus is a chronic, life-threatening illness that affects people of every age and ethnicity. It is a long-suffering pain for those who are affected and must regulate their blood glucose level by frequent subcutaneous injection of insulin every day. Herein, we propose a non-insulin and antidiabetic-drug-free strategy for regulating blood glucose level by a nano-sized "sugar sponge" which is a lectin-bound glycopolymersome capable of regulating glucose due to the dynamic recognition between the lectin and different carbohydrates...
May 16, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28502807/augmenting-brain-metabolism-to-increase-macro-and-chaperone-mediated-autophagy-for-decreasing-neuronal-proteotoxicity-and-aging
#3
REVIEW
Ben Loos, Daniel J Klionsky, Esther Wong
Accumulation of toxic protein aggregates in the nerve cells is a hallmark of neuronal diseases and brain aging. Mechanisms to enhance neuronal surveillance to improve neuronal proteostasis have a direct impact on promoting neuronal health and forestalling age-related decline in brain function. Autophagy is a lysosomal degradative pathway pivotal for neuronal protein quality control. Different types of autophagic mechanisms participate in protein handling in neurons. Macroautophagy targets misfolded and aggregated proteins in autophagic vesicles to the lysosomes for destruction, while chaperone-mediated autophagy (CMA) degrades specific soluble cytosolic proteins delivered to the lysosomes by chaperones...
May 11, 2017: Progress in Neurobiology
https://www.readbyqxmd.com/read/28480340/robot-assisted-radical-prostatectomy-has-lower-biochemical-recurrence-than-laparoscopic-radical-prostatectomy-systematic-review-and-meta-analysis
#4
Seon Heui Lee, Hyun Ju Seo, Na Rae Lee, Soo Kyung Son, Dae Keun Kim, Koon Ho Rha
PURPOSE: To assess the effectiveness and safety of robot-assisted radical prostatectomy (RARP) versus laparoscopic radical prostatectomy (LRP) in the treatment of prostate cancer. MATERIALS AND METHODS: Existing systematic reviews were updated to investigate the effectiveness and safety of RARP. Electronic databases, including Ovid MEDLINE, Ovid Embase, the Cochrane Library, KoreaMed, Kmbase, and others, were searched through July 2014. The quality of the selected systematic reviews was assessed by using the revised assessment of multiple systematic reviews (R-Amstar) and the Cochrane Risk of Bias tool...
May 2017: Investigative and Clinical Urology
https://www.readbyqxmd.com/read/28475226/molecular-cytogenetic-characterization-of-five-f8-complex-rearrangements-utility-for-haemophilia-a-genetic-counselling
#5
Y Jourdy, N Chatron, M Fretigny, M L Carage, H Chambost, S Claeyssens-Donadel, V Roussel-Robert, C Negrier, D Sanlaville, C Vinciguerra
BACKGROUND: Genomic inversions are usually balanced, but unusual patterns have been described in haemophilia A (HA) patients for intron 22 (Inv22) and intron 1 (Inv1) inversions leading to the hypothesis of more complex rearrangements involving deletions or duplications. AIM: To characterize five abnormal patterns either in Southern blot and long-range PCR for Inv22 or in PCR for Inv1. MATERIALS AND METHODS: All patients were studied using cytogenetic microarray analysis (CMA)...
May 5, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28475021/individualized-3d-printed-model-assisted-posterior-screw-fixation-for-the-treatment-of-craniovertebral-junction-abnormality-a-retrospective-study
#6
Fangyou Gao, Qu Wang, Chuangxi Liu, Bing Xiong, Tao Luo
OBJECTIVE This study was undertaken to evaluate the feasibility and efficacy of individualized 3D printed model-assisted posterior internal fixation in treating craniovertebral junction (CVJ) abnormalities. METHODS Forty-four patients (19 males and 25 females; mean age 36.5 ± 9.2 years, range 11-62 years; symptom duration 1 month-15 years) with a CVJ abnormality who were admitted to the authors' institution between April 2010 and April 2015 were retrospectively studied. The individualized 3D printed model of the CVJ was produced based on the individual CT data by use of 3D printing technology...
May 5, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28472932/chromosome-microarray-analysis-in-the-investigation-of-children-with-congenital-heart-disease
#7
Xiao-Li Wu, Ru Li, Fang Fu, Min Pan, Jin Han, Xin Yang, Yong-Ling Zhang, Fa-Tao Li, Can Liao
BACKGROUND: Our study was aimed to explore the clinical implication of chromosome microarray analysis (CMA) in genetically etiological diagnosis of children with congenital heart disease (CHD). METHODS: A total of 104 children with CHD with or without multiple congenital anomalies (MCA) or intellectual disabilities/developmental delay (ID/DD) but normal karyotype were investigated using Affymetrix CytoScan HD array. RESULT: Pathogenic copy number variations (PCNVs) were identified in 29 children (27...
May 4, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28471434/a-microcosting-and-cost-consequence-analysis-of-clinical-genomic-testing-strategies-in-autism-spectrum-disorder
#8
Kate Tsiplova, Richard M Zur, Christian R Marshall, Dimitri J Stavropoulos, Sergio L Pereira, Daniele Merico, Edwin J Young, Wilson W L Sung, Stephen W Scherer, Wendy J Ungar
PurposeWhole-exome (WES) and whole-genome sequencing (WGS) increase the diagnostic yield in autism spectrum disorder (ASD) compared to chromosomal microarray (CMA), but there have been no comprehensive cost analyses. The objective was to perform such an assessment of CMA, WES, and WGS and compare the incremental cost per additional positive finding in hypothetical testing scenarios.MethodsFive-year patient and program costs were estimated from an institutional perspective. WES and WGS estimates were based on HiSeq 2500 with an additional WGS estimate for HiSeq X platforms...
May 4, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28469644/the-emerging-role-of-autophagic-lysosomal-dysfunction-in-gaucher-disease-and-parkinson-s-disease
#9
REVIEW
Kerri J Kinghorn, Amir M Asghari, Jorge Iván Castillo-Quan
Gaucher disease (GD), the commonest lysosomal storage disorder, results from the lack or functional deficiency of glucocerebrosidase (GCase) secondary to mutations in the GBA1 gene. There is an established association between GBA1 mutations and Parkinson's disease (PD), and indeed GBA1 mutations are now considered to be the greatest genetic risk factor for PD. Impaired lysosomal-autophagic degradation of cellular proteins, including α-synuclein (α-syn), is implicated in the pathogenesis of PD, and there is increasing evidence for this also in GD and GBA1-PD...
March 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28465723/a-case-with-concurrent-duplication-triplication-and-uniparental-isodisomy-at-1q42-12-qter-supporting-microhomology-mediated-break-induced-replication-model-for-replicative-rearrangements
#10
Tomohiro Kohmoto, Nana Okamoto, Takuya Naruto, Chie Murata, Yuya Ouchi, Naoko Fujita, Hidehito Inagaki, Shigeko Satomura, Nobuhiko Okamoto, Masako Saito, Kiyoshi Masuda, Hiroki Kurahashi, Issei Imoto
BACKGROUND: Complex genomic rearrangements (CGRs) consisting of interstitial triplications in conjunction with uniparental isodisomy (isoUPD) have rarely been reported in patients with multiple congenital anomalies (MCA)/intellectual disability (ID). One-ended DNA break repair coupled with microhomology-mediated break-induced replication (MMBIR) has been recently proposed as a possible mechanism giving rise to interstitial copy number gains and distal isoUPD, although only a few cases providing supportive evidence in human congenital diseases with MCA have been documented...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/28465352/cystinosin-the-small-gtpase-rab11-and-the-rab7-effector-rilp-regulate-intracellular-trafficking-of-the-chaperone-mediated-autophagy-receptor-lamp2a
#11
Jinzhong Zhang, Jennifer L Johnson, Jing He, Gennaro Napolitano, Mahalakshmi Ramadass, Celine Rocca, William B Kiosses, Cecilia Bucci, Qisheng Xin, Evripidis Gavathiotis, Ana María Cuervo, Stephanie Cherqui, Sergio D Catz
The lysosomal storage disease cystinosis, caused by cystinosin-deficiency, is characterized by cell malfunction, tissue failure and progressive renal injury despite cystine-depletion therapies. Cystinosis is associated with defects in chaperone-mediated autophagy (CMA), but the molecular mechanisms are incompletely understood. Here, we show CMA substrate accumulation in cystinotic kidney proximal tubule cells. We also found mislocalization of the CMA lysosomal receptor LAMP2A, and impaired substrate translocation into the lysosome caused by defective CMA in cystinosis...
May 2, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28459985/cow-s-milk-allergy-in-infancy-and-later-development-of-juvenile-idiopathic-arthritis-a-register-based-case-control-study
#12
Miika Arvonen, J Lauri, Virta, Tytti Pokka, Liisa Kröger, Paula Vähäsalo
We have examined the association between cow's milk allergy (CMA) and juvenile idiopathic arthritis (JIA). The material for this case-control-study was collected from national registers of all children born between 2000 and 2010 in Finland and diagnosed with JIA (n = 1,298) and age-, gender- and place-matched controls (n = 5,179). We identified 235 children with CMA, 66 of these also had JIA. A conditional logistic regression analysis was performed to evaluate the association between CMA and JIA and to test whether exposure to antibiotics would be a covariate for this association...
April 28, 2017: American Journal of Epidemiology
https://www.readbyqxmd.com/read/28449395/breast-milk-iga-to-foods-has-different-epitope-specificity-than-serum-iga-evidence-for-entero-mammary-link-for-food-specific-iga
#13
Antti E Seppo, Emma M Savilahti, M Cecilia Berin, Hugh A Sampson, Kirsi M Järvinen
BACKGROUND: We have previously shown that maternal cow's milk (CM) elimination results in down-regulation of CM-specific IgA antibody levels in BM, but not in serum, suggesting that an entero-mammary link may exist for food-specific antibody-secreting cells. OBJECTIVE: We sought to investigate whether food-specific IgA epitope profiles differ intra-individually between mother's serum and BM. We also examined how infants' food epitope-specific IgA develops in early infancy and the relationship of IgA epitope recognition with development of cow's milk allergy (CMA)...
April 27, 2017: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28449382/current-use-of-chromosomal-microarray-by-australian-paediatricians-and-implications-for-the-implementation-of-next-generation-sequencing
#14
Victoria McKay, Daryl Efron, Elizabeth E Palmer, Susan M White, Chris Pearson, Margie Danchin
AIM: Chromosomal microarray (CMA) is an important diagnostic test for children with multiple congenital anomalies or certain developmental behavioural problems suggestive of an underlying genetic diagnosis. However, there are medical and ethical complexities to its use and few Australian policies to guide practice. We aimed to describe the current practice of Australian paediatricians in relation to CMA testing. We hypothesised that there are knowledge gaps in their use of CMA. METHODS: Online survey completed between September 2015 and January 2016 by paediatricians in secondary care settings...
April 27, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28428740/protein-quality-control-by-molecular-chaperones-in-neurodegeneration
#15
REVIEW
Aaron Ciechanover, Yong Tae Kwon
Protein homeostasis (proteostasis) requires the timely degradation of misfolded proteins and their aggregates by protein quality control (PQC), of which molecular chaperones are an essential component. Compared with other cell types, PQC in neurons is particularly challenging because they have a unique cellular structure with long extensions. Making it worse, neurons are postmitotic, i.e., cannot dilute toxic substances by division, and, thus, are highly sensitive to misfolded proteins, especially as they age...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28428710/cma-down-regulates-p53-expression-through-degradation-of-hmgb1-protein-to-inhibit-irradiation-triggered-apoptosis-in-hepatocellular-carcinoma
#16
Jing-Hua Wu, Jia-Pei Guo, Jun Shi, Hui Wang, Lei-Lei Li, Bin Guo, Dian-Xing Liu, Qing Cao, Zhi-Yong Yuan
AIM: To investigate the mechanism of chaperone-mediated autophagy (CMA)-induced resistance to irradiation-triggered apoptosis through regulation of the p53 protein in hepatocellular carcinoma (HCC). METHODS: Firstly, we detected expression of lysosome-associated membrane protein 2a (Lamp-2a), which is the key protein of CMA, by western blot in HepG2 and SMMC7721 cells after irradiation. We further used shRNA Lamp-2a HCC cells to verify the radioresistance induced by CMA...
April 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28424868/non-invasive-measurements-of-the-dynamic-changes-in-the-ciliary-muscle-crystalline-lens-morphology-and-anterior-chamber-during-accommodation-with-a-high-resolution-oct
#17
José J Esteve-Taboada, Alberto Domínguez-Vicent, Daniel Monsálvez-Romín, Antonio J Del Águila-Carrasco, Robert Montés-Micó
PURPOSE: The purpose of the study was to assess non-invasively the changes in the anterior chamber eye, crystalline lens morphology, and ciliary muscle during accommodation by means of an anterior chamber optical coherence tomographer (OCT), and correlate them with vergence. METHODS: Twenty-five eyes of twenty-five healthy subjects, whose mean age was 29.9±7.1 years, were included and measured with an anterior chamber OCT. The central corneal thickness (CCT), anterior chamber depth (ACD), anterior crystalline lens radius of curvature (ALRC), crystalline lens thickness (CLT), and ciliary muscle area (CMA) were measured for each participant at 0, -1, -2, and -3 D of target vergence...
April 20, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/28417624/cow-s-milk-desensitization-in-anaphylactic-patients-a-new-personalized-dose-method
#18
Delara Babaie, Mohammad Nabavi, Saba Arshi, Mehrnaz Mesdaghi, Zahra Chavoshzadeh, Mohammad Hasan Bemanian, Mitra Tafakori, Mehrdad Amirmoini, Hosein Esmailzadeh, Rasoul Molatefi, Mahsa Rekabi, Nadieh Akbarpour, Farimah Masoumi, Morteza Fallahpour
Cow's milk allergy (CMA) is the most frequent food allergy in children and oral immunotherapy (OIT) is a promising approach for treatment of patients. The most challenging cases are anaphylactic with coexisting asthma and proposing safe protocols is crucial especially in high risk groups. Considering that CMA varies among patients, an individualized OIT protocol would be beneficial to achieve a safer and more efficient method of desensitization. 18 children more than 3 years of age with IgE-mediated CMA were enrolled...
February 2017: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/28416249/prognostic-accuracy-of-clinical-signs-and-diagnostic-tests-in-cow-s-milk-allergy-in-newborns
#19
Gregor Nosan, Maja Jakic, Miha Jager, Darja Paro-Panjan
BACKGROUND: The aim of the study was to explore the correlation between clinical signs and confirmatory tests for cow's milk allergy (CMA) in the neonatal period and their relation to family history and the occurrence of food allergies in the postneonatal period. METHODS: The medical documentation of 361 newborns with suspected CMA and exclusion of other comorbidities was analyzed. The correlations between clinical signs and methods to confirm CMA [elevated levels of total immunoglobulin E (IgE) and/or specific IgE for cow's milk, improvement after the introduction of a cow's milk-free diet and positive challenge procedure] were studied...
March 27, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28410006/chaperone-mediated-autophagy-prevents-cellular-transformation-by-regulating-myc-proteasomal-degradation
#20
Luciana R Gomes, Carlos F M Menck, Ana Maria Cuervo
Chaperone-mediated autophagy (CMA), a selective form of protein lysosomal degradation, is maximally activated in stress situations to ensure maintenance of cellular homeostasis. CMA activity decreases with age and in several human chronic disorders, but in contrast, in most cancer cells, CMA is upregulated and required for tumor growth. However, the role of CMA in malignant transformation remains unknown. In this study, we demonstrate that CMA inhibition in fibroblasts augments the efficiency of MYC/c-Myc-driven cellular transformation...
March 2, 2017: Autophagy
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