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https://www.readbyqxmd.com/read/29667913/ubiquitin-c-terminal-hydrolase-l1-deletion-is-associated-with-urinary-%C3%AE-klotho-deficiency-and-perturbed-phosphate-homeostasis
#1
Naomi C Boisvert, Chet E Holterman, Alexey Gutsol, Josee Coulombe, Wanling Pan, R Todd Alexander, Douglas A Gray, Chris R Kennedy
Loss of ubiquitin C-terminal hydrolase L1 (UCHL1), a deubiquitinating enzyme required for neuronal function, led to hyperphosphatemia accompanied by phosphaturia in mice, while calcium homeostasis remained intact. We therefore investigated the mechanisms underlying the phosphate imbalance in Uchl1-/- mice. Interestingly, phosphaturia was not a result of lower renal brush border membrane sodium-phosphate cotransporter expression as sodium-phosphate cotransporter 2a and 2c expression levels were similar to wild type levels...
April 18, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29658965/a-comprehensive-transcriptome-analysis-of-skeletal-muscles-in-two-polish-pig-breeds-differing-in-fat-and-meat-quality-traits
#2
Katarzyna Piórkowska, Kacper Żukowski, Katarzyna Ropka-Molik, Mirosław Tyra, Artur Gurgul
Pork is the most popular meat in the world. Unfortunately, the selection pressure focused on high meat content led to a reduction in pork quality. The present study used RNA-seq technology to identify metabolic process genes related to pork quality traits and fat deposition. Differentially expressed genes (DEGs) were identified between pigs of Pulawska and Polish Landrace breeds for two the most important muscles (semimembranosus and longissimus dorsi). A total of 71 significant DEGs were reported: 15 for longissimus dorsi and 56 for semimembranosus muscles...
January 2018: Genetics and Molecular Biology
https://www.readbyqxmd.com/read/29627846/upregulation-of-ubiquitin-carboxyl-terminal-hydrolase-l1-uchl1-mediates-the-reversal-effect-of-verapamil-on-chemo-resistance-to-adriamycin-of-hepatocellular-carcinoma
#3
Guangshan Yang, Gaofei Fan, Tengyue Zhang, Kelong Ma, Jin Huang, Miao Liu, Xiaolu Teng, Kun Xu, Pingsheng Fan, Dongmiao Cheng
BACKGROUND The aim of this study was to investigate the role of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) in the reversal effect of verapamil (VER) on chemo-resistance to Adriamycin (ADM) in treatment of hepatocellular carcinoma (HCC). MATERIAL AND METHODS HCC cell lines SMMC-7721 and BEL-7402 were used as model cell lines. High-throughput transcriptome sequencing based on Illumina technology was used to screen whether UCHL1 mediated the reversal effect of VER on chemo-resistance. Quantitative real-time PCR (qRT-PCR) was performed to determine the expression level of UCHL1 mRNA in HCC cells, and western blot analysis was performed to examine the protein expression of UCHL1 protein in HCC cells...
April 8, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29539261/extracellular-%C3%AE-synuclein-disrupts-membrane-nanostructure-and-promotes-s-nitrosylation-induced-neuronal-cell-death
#4
Roshan Kumar, Raniki Kumari, Sanjay Kumar, Deepak Kumar Jangir, Tushar Kanti Maiti
α-Synuclein, a major constituent of proteinaceous inclusions named Lewy body, has been shown to be released and taken up by cells, which may facilitate its progressive pathological spreading and neuronal cell death in Parkinson's disease. However, the pathophysiological effect and signalling cascade initiated by extracellular α-synuclein in cellular milieu are not well understood. Herein we have investigated the perturbations induced by low molecular weight α-synuclein and different types of α-synuclein oligomers in the neuroblastoma SH-SY5Y cells...
March 14, 2018: Biomacromolecules
https://www.readbyqxmd.com/read/29535397/drosophila-ubiquitin-c-terminal-hydrolase-knockdown-model-of-parkinson-s-disease
#5
Hiep H Tran, Suong N A Dang, Thanh T Nguyen, Anh M Huynh, Linh M Dao, Kaeko Kamei, Masamitsu Yamaguchi, Thao T P Dang
Parkinson's disease (PD) is the second most common neurodegenerative disorder worldwide. Many factors have been shown to contribute to its pathogenesis including genetic and environmental factors. Ubiquitin C-terminal hydrolase L1 (UCHL1) is also known to be involved in the pathogenesis of PD. We herein modeled the study of UCHL1 in Drosophila melanogaster and investigated its functions in PD. The specific knockdown of the Drosophila ortholog of UCHL1 (dUCH) in dopaminergic neurons (DA neurons) led to the underdevelopment and/or degeneration of these neurons, specifically in DL1 DA neuron cluster in the larval brain lobe and PPM2, PPM3, PPL2ab, and VUM DA neuron clusters in the adult brain...
March 13, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29534191/immunoproteasome-in-the-plasma-of-paediatric-patients-with-moderate-and-major-burns-and-its-correlation-with-proteasome-and-uchl1-measured-by-spr-imaging-biosensors
#6
Ewa Matuszczak, Artur Weremijewicz, Marta Komarowska, Anna Sankiewicz, Diana Markowska, Wojciech Debek, Ewa Gorodkiewicz, Robert Milewski, Adam Hermanowicz
THE AIM: to determinate the immunoproteasome concentration in blood plasma of paediatric patients with moderate and major burns, and its correlation with circulating proteasome and UCHL1 with SPRI biosensor. MATERIAL AND METHODS: The study population comprised 30 patients with moderate (n=21), and severe burns (n=9), aged 9 months to 14 years. The control group represented 18 healthy, age matched patients, admitted for herniotomy. Exclusion criteria were: admission to the hospital later than 6 hours after burn, cardiovascular or immunological diseases, and severe preexisting infections...
March 9, 2018: Journal of Burn Care & Research: Official Publication of the American Burn Association
https://www.readbyqxmd.com/read/29523900/relationships-between-markers-of-neurologic-and-endothelial-injury-during-critical-illness-and-long-term-cognitive-impairment-and-disability
#7
Christopher G Hughes, Mayur B Patel, Nathan E Brummel, Jennifer L Thompson, J Brennan McNeil, Pratik P Pandharipande, James C Jackson, Rameela Chandrasekhar, Lorraine B Ware, E Wesley Ely, Timothy D Girard
PURPOSE: Neurologic and endothelial injury biomarkers are associated with prolonged delirium during critical illness and may reflect injury pathways that lead to poor long-term outcomes. We hypothesized that blood-brain barrier (BBB), neuronal, and endothelial injury biomarkers measured during critical illness are associated with cognitive impairment and disability after discharge. METHODS: We enrolled adults with respiratory failure and/or shock and measured plasma concentrations of BBB (S100B), neuronal (UCHL1, BDNF), and endothelial (E-selectin, PAI-1) injury markers within 72 h of ICU admission...
March 9, 2018: Intensive Care Medicine
https://www.readbyqxmd.com/read/29489474/high-expression-of-ubiquitin-c-terminal-hydrolase-l1-is-associated-with-poor-prognosis-in-endometrial-cancer-patients
#8
Kohshiro Nakao, Takashi Hirakawa, Hiroto Suwa, Kayoko Kogure, Sadatomo Ikeda, Soichi Yamashita, Takashi Minegishi, Hiroshi Kishi
OBJECTIVE: The ubiquitin C-terminal hydrolase L1 (UCHL1) plays a key role in tumor invasion and metastasis. Ubiquitin C-terminal hydrolase L1 is overexpressed in various cancers and reported to be correlated with a poor prognosis. The objective of this study was to determine the prognostic significance of UCHL1 in endometrial cancer. METHODS: The expression of UCHL1 in endometrial cancer was assessed using quantitative reverse transcription polymerase chain reaction and immunohistochemistry in 56 and 215 resected tumor specimens, respectively...
February 28, 2018: International Journal of Gynecological Cancer
https://www.readbyqxmd.com/read/29462615/ubiquitin-c-terminal-hydrolase-l1-regulates-autophagy-by-inhibiting-autophagosome-formation-through-its-deubiquitinating-enzyme-activity
#9
Cong Yan, Huanhuan Huo, Cuiwei Yang, Tao Zhang, Yuanyuan Chu, Yanfen Liu
Ubiquitination modification has been shown to play a key role in autophagy. Increasing studies reported the involvement of de-ubiquitinating enzymes (DUBs) in autophagy pathway. To systematically search how DUBs manipulate autophagy, we utilized a double fluorescence tagged LC3 stable HeLa cell line, and did a genome wide screen of 55 human DUBs which is about 60% coverage of the DUB family. We found a bunch of DUBs have impact on autophagy by either changing the LC3 puncta formation or the autophagy flux. One of them, Ubiquitin C-Terminal Hydrolase L1 (UCHL1) correlated to Parkinson's disease, strongly affects autophagy by inhibiting autophagosome formation...
February 17, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29453387/structural-determinants-of-the-sine-b2-element-embedded-in-the-long-non-coding-rna-activator-of-translation-as-uchl1
#10
Peter Podbevšek, Francesca Fasolo, Carlotta Bon, Laura Cimatti, Sabine Reißer, Piero Carninci, Giovanni Bussi, Silvia Zucchelli, Janez Plavec, Stefano Gustincich
Pervasive transcription of mammalian genomes leads to a previously underestimated level of complexity in gene regulatory networks. Recently, we have identified a new functional class of natural and synthetic antisense long non-coding RNAs (lncRNA) that increases translation of partially overlapping sense mRNAs. These molecules were named SINEUPs, as they require an embedded inverted SINE B2 element for their UP-regulation of translation. Mouse AS Uchl1 is the representative member of natural SINEUPs. It was originally discovered for its role in increasing translation of Uchl1 mRNA, a gene associated with neurodegenerative diseases...
February 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29401475/overexpression-of-ubiquitin-carboxyl-terminal-hydrolase-1-uchl1-in-boys-with-cryptorchidism
#11
Dorota Toliczenko-Bernatowicz, Ewa Matuszczak, Marzena Tylicka, Beata Szymańska, Marta Komarowska, Ewa Gorodkiewicz, Wojciech Debek, Adam Hermanowicz
BACKGROUND: The ubiquitin-proteasome system regulate p53, caspase and Bcl-2 family proteins, and is crucial for the degradation of the defective germ cells in testes. Purpose: to evaluate the concentration of ubiquitin carboxyl-terminal hydrolase 1 (UCHL1) in the blood plasma of boys with cryptorchidism and if there is any correlation with patient age. METHODS: Patients-50 boys aged 1-4 years (median = 2,4y.) with unilateral cryptorchidism. Exclusion criteria were: previous human chorionic gonadotropin treatment, an abnormal karyotype, endocrine or immunological disorders or any long-term medication...
2018: PloS One
https://www.readbyqxmd.com/read/29397366/rare-disease-mechanisms-identified-by-genealogical-proteomics-of-copper-homeostasis-mutant-pedigrees
#12
Stephanie A Zlatic, Alysia Vrailas-Mortimer, Avanti Gokhale, Lucas J Carey, Elizabeth Scott, Reid Burch, Morgan M McCall, Samantha Rudin-Rush, John Bowen Davis, Cortnie Hartwig, Erica Werner, Lian Li, Michael Petris, Victor Faundez
Rare neurological diseases shed light onto universal neurobiological processes. However, molecular mechanisms connecting genetic defects to their disease phenotypes are elusive. Here, we obtain mechanistic information by comparing proteomes of cells from individuals with rare disorders with proteomes from their disease-free consanguineous relatives. We use triple-SILAC mass spectrometry to quantify proteomes from human pedigrees affected by mutations in ATP7A, which cause Menkes disease, a rare neurodegenerative and neurodevelopmental disorder stemming from systemic copper depletion...
January 30, 2018: Cell Systems
https://www.readbyqxmd.com/read/29359783/aberrant-methylation-of-htatip2-and-uchl1-as-a-predictive-biomarker-for-cholangiocarcinoma
#13
Chaiyachet Nanok, Patcharee Jearanaikoon, Siriporn Proungvitaya, Temduang Limpaiboon
Cholangiocarcinoma (CCA) is the most common primary liver cancer in Northeastern Thailand where liver fluke infection is highly endemic. Although aberrant DNA methylation in CCA has been reported by several investigators, little is known regarding the associations between them. In the present study, the results obtained from our previously published methylation array were analyzed and 10 candidate genes involved in DNA repair [protein phosphatase 4 catalytic subunit (PPP4C)], apoptosis [runt related transcription factor 3 (RUNX3), interferon regulatory factor 4 (IRF4), ubiquitin C‑terminal hydrolase L1 (UCHL1) and tumor protein p53 inducible protein 3 (TP53I3)], cell proliferation [cyclin D2 (CCND2) and Ras association domain family member 1 (RASSF1)], drug metabolism [aldehyde dehydrogenase 1 family member A3 (ALDH1A3) and solute carrier family 29 member 1 (SLC29A1)] and angiogenesis [human immunodeficiency virus‑1 tat interactive protein 2 (HTATIP2)] were selected for quantification of their methylation levels in 54 CCA and 19 adjacent normal tissues using methylation‑sensitive high‑resolution melting...
December 19, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29285276/pilot-study-molecular-risk-factors-for-diagnosing-sporadic-parkinson-s-disease-based-on-gene-expression-in-blood-in-mptp-induced-rhesus-monkeys
#14
Liangqin Shi, Chao Huang, Qihui Luo, Yu Xia, Heng Liu, Like Li, Wentao Liu, Wenjing Ma, Jing Fang, Li Tang, Wen Zeng, Zhengli Chen
Clinical diagnosis of Parkinson's disease (PD) is characterized by the classical features of tremor, bradykinesia and rigidity, which are present only when more than 70%-80% degeneration of dopaminergic (DA) neurons in the substantia nigra. The lack of means for early diagnosis of PD has elicited interest in searching for its risk factors, which, by now, are almost obtained at a single time point in PD process, and little developing risk factors, obtained from completely normal situation to the onset or even advanced stage of PD in individual person which could monitor the progress of PD, are present...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29277369/implication-of-altered-ubiquitin-proteasome-system-and-er-stress-in-the-muscle-atrophy-of-diabetic-rats
#15
S Sreenivasa Reddy, Karnam Shruthi, Y Konda Prabhakar, Gummadi Sailaja, G Bhanuprakash Reddy
BACKGROUND: Skeletal muscle is adversely affected in type-1 diabetes, and excessively stimulated ubiquitin-proteasome system (UPS) was found to be a leading cause of muscle wasting or atrophy. The role of endoplasmic reticulum (ER) stress in muscle atrophy of type-1 diabetes is not known. Hence, we investigated the role of UPS and ER stress in the muscle atrophy of chronic diabetes rat model. METHODS: Diabetes was induced with streptozotocin (STZ) in male Sprague-Dawley rats and were sacrificed 2- and 4-months thereafter to collect gastrocnemius muscle...
February 1, 2018: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/29241710/new-insights-into-the-gene-expression-associated-to-amyotrophic-lateral-sclerosis
#16
REVIEW
Daniela Recabarren-Leiva, Marcelo Alarcón
Amyotrophic lateral sclerosis (ALS) is the most prevalent neuromuscular disease worldwide. It is a lethal and progressive neurodegenerative disease, principally affecting motor neurons; patient clinical characteristics are muscle weakness, dysphagia and respiratory failure. The mean age is related to family history (40years, familial ALS or FALS) or with no family history (50years), but it is more common in people aged 60-69years. The cause of ALS is not known and it is not known yet why it affects some people and not others...
January 15, 2018: Life Sciences
https://www.readbyqxmd.com/read/29156776/uchl1-expression-and-localization-on-testicular-development-and-spermatogenesis-of-chinese-giant-salamanders
#17
Yuanxian Wang, Liqing Wang, Huihui Gao, Yao Gao, Changming Yang, Hong Ji, Wuzi Dong
Ubiquitin carboxyl-terminal hydrolase L1 (UCHL1), which is extensively expressed in vertebrates, is a deubiquitinating enzymes that inhibits the degradation of proteins by reversing ubiquitination modification. Herein, a 1087-bp sequence encoding UCHL1 was identified from the Chinese giant salamander (CGS; Andrias davidianus ). The coding sequences (CDS) of UCHL1 encoded a putative poly peptide of 222 amino acids. The CGS UCHL1 isoforms were more related to their human and mouse counterparts. The phylogenic tree of vertebrate UCHL1 indicated that CGS UCHL1 has the closest relationship with human UCHL1 (up to 73...
October 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29156418/characterization-of-domestic-pig-spermatogenesis-using-spermatogonial-stem-cell-markers-in-the-early-months-of-life
#18
Julio Almunia, Kotono Nakamura, Mami Murakami, Shigeo Takashima, Masaki Takasu
In pigs, spermatogonial stem cells (SSCs), which include gonocytes and undifferentiated spermatogonia, are classically defined as being present till up to 2 months of life. However, knowledge about this transition from gonocytes to undifferentiated spermatogonia in pigs is limited. In this study, we characterized pig SSCs in neonatal (n = 6), one-month-old (1-mo-old, n = 6), two-month-old (2-mo-old, n = 6), and adult testes (n = 6) anatomically, histologically, and immunohistologically. Anatomically, testicular circumference (TC) was measured at each development stage...
February 2018: Theriogenology
https://www.readbyqxmd.com/read/29150024/decreased-uchl1-expression-as-a-cytologic-biomarker-for-aggressive-behavior-in-pancreatic-neuroendocrine-tumors
#19
Maureen D Moore, Brendan Finnerty, Katherine D Gray, Rana Hoda, Yi-Fan Liu, Lauren Soong, Toni Beninato, Rema Rao, Rasa Zarnegar, Thomas J Fahey
BACKGROUND: There are currently no reliable markers associated with aggressive behavior in well-differentiated and moderately differentiated pancreatic neuroendocrine tumors. We aimed to determine whether expression of ubiquitin carboxyl-terminal hydrolase L1 in conjunction with Ki67 can identify metastatic potential of well-differentiated and moderately differentiated pancreatic neuroendocrine tumors from fine-needle aspiration samples obtained by endoscopic ultrasound. METHODS: Retrospective review of 48 patients with well-differentiated and moderately differentiated pancreatic neuroendocrine tumors diagnosed by endoscopic ultrasound fine-needle aspiration at a single center identified 35 biopsy samples with adequate material for analysis...
January 2018: Surgery
https://www.readbyqxmd.com/read/29126443/ubiquitin-c-terminal-hydrolase-isozyme-l1-is-associated-with-shelterin-complex-at-interstitial-telomeric-sites
#20
Aleksandar Ilic, Sumin Lu, Vikram Bhatia, Farhana Begum, Thomas Klonisch, Prasoon Agarwal, Wayne Xu, James R Davie
BACKGROUND: Ubiquitin C-terminal hydrolase isozyme L1 (UCHL1) is primarily expressed in neuronal cells and neuroendocrine cells and has been associated with various diseases, including many cancers. It is a multifunctional protein involved in deubiquitination, ubiquitination and ubiquitin homeostasis, but its specific roles are disputed and still generally undetermined. RESULTS: Herein, we demonstrate that UCHL1 is associated with genomic DNA in certain prostate cancer cell lines, including DU 145 cells derived from a brain metastatic site, and in HEK293T embryonic kidney cells with a neuronal lineage...
November 10, 2017: Epigenetics & Chromatin
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