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https://www.readbyqxmd.com/read/29777184/genotype-phenotype-links-in-frontotemporal-lobar-degeneration
#1
REVIEW
Sara Van Mossevelde, Sebastiaan Engelborghs, Julie van der Zee, Christine Van Broeckhoven
Frontotemporal lobar degeneration (FTLD) represents a group of neurodegenerative brain diseases with highly heterogeneous clinical, neuropathological and genetic characteristics. This high degree of heterogeneity results from the presence of several different underlying molecular disease processes; consequently, it is unlikely that all patients with FTLD will benefit from a single therapy. Therapeutic strategies for FTLD are currently being explored, and tools are urgently needed that enable the selection of patients who are the most likely to benefit from a particular therapy...
May 18, 2018: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/29770363/the-multifaceted-clinical-presentation-of-vcp-proteinopathy-in-a-greek-family
#2
George K Papadimas, George P Paraskevas, Thomas Zambelis, Chrisostomos Karagiaouris, Mara Bourbouli, Anastasia Bougea, Maggie C Walter, Nicolas U Schumacher, Sabine Krause, Elisabeth Kapaki
VCP-proteinopathy is a multisystem neurodegenerative disorder caused by mutations in valosin containing protein. Here, we report the first Greek case of VCP-proteinopathy in a 62 year old patient with a slowly progressing muscular weakness since his mid-40s and a severe deterioration during the last year. He also manifested dementia with prominent neuropsychiatric symptoms, including aggression, apathy, palilalia and obsessions. Brain MRI revealed frontal atrophy, while muscle MRI showed diffuse muscle atrophy...
December 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29768310/examining-women-veteran-s-experiences-perceptions-and-challenges-with-the-veterans-choice-program-vcp
#3
Kristin M Mattocks, Elizabeth M Yano, Amber Brown, Jose Casares, Lori Bastian
BACKGROUND: The Veterans Choice Program (VCP) was launched in 2014 to address the growing concerns about the timeliness and quality of Veterans Health Administration (VHA) care. Given that many sex-specific health services, such as mammography and maternity care, are not routinely provided in all VHA facilities, women Veterans may disproportionately rely on VCP care. Understanding the provision and coordination of VCP care is crucial in order to ensure that care is not fragmented across the 2 health care systems...
May 15, 2018: Medical Care
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#4
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29754758/novel-valosin-containing-protein-mutations-associated-with-multisystem-proteinopathy
#5
Sejad Al-Tahan, Ebaa Al-Obeidi, Hiroshi Yoshioka, Anita Lakatos, Lan Weiss, Marjorie Grafe, Johanna Palmio, Matt Wicklund, Yadollah Harati, Molly Omizo, Bjarne Udd, Virginia Kimonis
Over fifty missense mutations in the gene coding for valosin-containing protein (VCP) are associated with a unique autosomal dominant adult-onset progressive disease associated with combinations of proximo-distal inclusion body myopathy (IBM), Paget's disease of bone (PDB), frontotemporal dementia (FTD), and amyotrophic lateral sclerosis (ALS). We report the clinical, histological, and molecular findings in four new patients/families carrying novel VCP mutations: c.474 G > A (p.M158I); c.478 G > C (p...
April 17, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29748747/use-of-guideline-recommended-treatments-for-ptsd-among-community-based-providers-in-texas-and-vermont-implications-for-the-veterans-choice-program
#6
Erin P Finley, Michael Mader, Elizabeth K Haro, Polly H Noël, Nancy Bernardy, Craig S Rosen, Mary Bollinger, Hector A Garcia, Kathleen Sherrieb, Mary Jo V Pugh
Implementation of the Veterans Choice Program (VCP) allows Veterans to receive care paid for by the Department of Veterans Affairs (VA) in community settings. However, the quality of that care is unknown, particularly for complex conditions such as posttraumatic stress disorder (PTSD). A cross-sectional survey was conducted of 668 community primary care and mental health providers in Texas and Vermont to describe use of guideline-recommended treatments (GRTs) for PTSD. Relatively, few providers reported using guideline-recommended psychotherapy or prescribing practices...
May 10, 2018: Journal of Behavioral Health Services & Research
https://www.readbyqxmd.com/read/29743287/ptpn12-ptp-pest-regulates-phosphorylation-dependent-ubiquitination-and-stability-of-focal-adhesion-substrates-in-invasive-glioblastoma-cells
#7
Zhihua Chen, John E Morales, Paola A Guerrero, Huandong Sun, Joseph H McCarty
Glioblastoma (GBM) is an invasive brain cancer with tumor cells that disperse from the primary mass, escaping surgical resection and invariably giving rise to lethal recurrent lesions. Here we report that PTP-PEST, a cytoplasmic protein tyrosine phosphatase, controls GBM cell invasion by physically bridging the focal adhesion protein Crk-associated substrate (Cas) to valosin containing protein (Vcp), an ATP-dependent protein segregase that selectively extracts ubiquitinated proteins from multiprotein complexes and targets them for degradation via the ubiquitin proteasome system...
May 9, 2018: Cancer Research
https://www.readbyqxmd.com/read/29719249/skeletal-muscle-specific-methyltransferase-mettl21c-trimethylates-p97-and-regulates-autophagy-associated-protein-breakdown
#8
Janica Lea Wiederstein, Hendrik Nolte, Stefan Günther, Tanja Piller, Martina Baraldo, Sawa Kostin, Wilhelm Bloch, Natalie Schindler, Marco Sandri, Bert Blaauw, Thomas Braun, Soraya Hölper, Marcus Krüger
Protein aggregates and cytoplasmic vacuolization are major hallmarks of multisystem proteinopathies (MSPs) that lead to muscle weakness. Here, we identify METTL21C as a skeletal muscle-specific lysine methyltransferase. Insertion of a β-galactosidase cassette into the Mettl21c mouse locus revealed that METTL21C is specifically expressed in MYH7-positive skeletal muscle fibers. Ablation of the Mettl21c gene reduced endurance capacity and led to age-dependent accumulation of autophagic vacuoles in skeletal muscle...
May 1, 2018: Cell Reports
https://www.readbyqxmd.com/read/29717916/risk-factors-associated-with-the-development-of-acquired-airway-disease-after-congenital-heart-surgery-a-retrospective-cohort-study
#9
Andrew J Matisoff, Pranathi Ari, David Zurakowski, Alexandra G Espinel, Nina Deutsch, Brian K Reilly
OBJECTIVE: In this single-center, retrospective review, we sought to determine the risk factors associated with the development of severe acquired airway disease (AAD; vocal cord paralysis [VCP] or subglottic stenosis [SGS]) in pediatric patients who had undergone surgery for congenital heart disease (CHD) with cardiopulmonary bypass. All patients who required surgical treatment for CHD using cardiopulmonary bypass at our institution between 2010 and 2015 were reviewed. We defined severe AAD as either clinically significant VCP, SGS, or both, requiring consultation with the otolaryngology (ENT) service for evaluation...
April 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29704548/toxicity-and-aggregation-of-the-polyglutamine-disease-protein-ataxin-3-is-regulated-by-its-binding-to-vcp-p97-in-drosophila-melanogaster
#10
Gorica Ristic, Joanna R Sutton, Kozeta Libohova, Sokol V Todi
Among the nine dominantly inherited, age-dependent neurodegenerative diseases caused by abnormal expansion in the polyglutamine (polyQ) repeat of otherwise unrelated proteins is Spinocerebellar Ataxia Type 3 (SCA3). SCA3 is caused by polyQ expansion in the deubiquitinase (DUB), ataxin-3. Molecular sequelae related to SCA3 remain unclear. Here, we sought to understand the role of protein context in SCA3 by focusing on the interaction between this DUB and Valosin-Containing Protein (VCP). VCP is bound directly by ataxin-3 through an arginine-rich area preceding the polyQ repeat...
April 25, 2018: Neurobiology of Disease
https://www.readbyqxmd.com/read/29693262/interleukin-6-induced-overexpression-of-valosin-containing-protein-vcp-p97-is-associated-with-androgen-independent-prostate-cancer-aipc-progression
#11
Divya Duscharla, Karthik Reddy Kami Reddy, Chandrashekhar Dasari, Supriya Bhukya, Ramesh Ummanni
Though Androgen deprivation therapy (ADT) is effective initially, numerous patients become resistant to it and develop castration resistant PCa (CRPC). Cytokines promotes ligand independent activation of AR. Interleukin-6 (IL-6) levels are elevated in CRPC patients and regulate AR activity. However, progression to CRPC is not fully understood. In this study, we analyzed differential protein expression in LNCaP cells treated with IL-6 using proteomics. Results revealed altered expression of 27 proteins and Valosin-containing protein (VCP)/p97 plays a predominant role in co-regulation of altered proteins...
April 25, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29685906/the-vcp-ubxn1-complex-mediates-triage-of-ubiquitylated-cytosolic-proteins-bound-to-the-bag6-complex
#12
Rakesh Ganji, Sirisha Mukkavalli, Flavio Somanji, Malavika Raman
A balance between protein synthesis and degradation is necessary to maintain cellular homeostasis. Failure to triage aberrant proteins may result in their accumulation and aggregation in the cytosol. The VCP-BAG6 complex facilitates a wide variety of ubiquitin-mediated quality control events at the ER; both prior to ER translocation as well as during ER associated degradation (ERAD). Yet, how ubiquitylated clients associated with BAG6 are recognized by VCP for proteasomal degradation is presently unknown. We have identified UBXN1 as the VCP adaptor in BAG6-dependent processes occurring prior to ER insertion but not during ERAD...
April 23, 2018: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/29655804/characterization-of-wdr20-a-new-regulator-of-the-erad-machinery
#13
Lin-Gao Ju, Xiang Lin, Dong Yan, Qing-Lan Li, Min Wu, Lian-Yun Li
ERAD is an important process of protein quality control that eliminates misfolded or unassembled proteins from ER. Before undergoing proteasome degradation, the misfolded proteins are dislocated from ER membrane into cytosol, which requires the AAA ATPase p97/VCP and its cofactor, the NPL4-UFD1 dimer. Here, we performed a CRISPR-based screen and identify many candidates for ERAD regulation. We further confirmed four proteins, FBOX2, TRIM6, UFL1 and WDR20, are novel regulators for ERAD. Then the molecular mechanism for WDR20 in ERAD is further characterized...
April 12, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29611091/risk-factors-for-weight-loss-1-year-after-esophagectomy-and-gastric-pull-up-for-esophageal-cancer
#14
Seong Yong Park, Dae Joon Kim, Jee Won Suh, Go Eun Byun
BACKGROUND: Loss of body weight is regarded as a marker of malnutrition after esophagectomy. This study investigated changes in body weight and risk factors for weight loss after esophagectomy for esophageal cancer. METHODS: We retrospectively reviewed records of 181 patients who underwent esophagectomy and gastric pull-up from 2012 to June 2016. Patients with operative mortality and recurrences were excluded. Percent change in body weight was defined as change in body weight (%) = (1-year body weight - preoperative body weight) × 100/preoperative body weight...
April 2, 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/29611059/new-developments-in-the-genetics-of-inclusion-body-myositis
#15
REVIEW
Kyla A Britson, Stephanie Y Yang, Thomas E Lloyd
PURPOSE OF REVIEW: Our goal is to review the recent literature pertaining to the genetics of sporadic inclusion body myositis (IBM). RECENT FINDINGS: In a study of 252 IBM patients, the class II MHC allele HLA-DRB1*03:01 showed the most significant association with IBM, and that risk could be largely attributed to amino acids within the peptide-binding pocket. Candidate gene sequencing identified rare missense variants in proteins regulating protein homeostasis including VCP and SQSTM1...
April 2, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29599289/interaction-between-the-aaa-atpase-p97-vcp-and-a-concealed-ubx-domain-in-the-copper-transporter-atp7a-is-associated-with-motor-neuron-degeneration
#16
Ling Yi, Stephen G Kaler
The copper-transporting ATPase ATP7A contains eight transmembrane domains and is required for normal human copper homeostasis. Mutations in the ATP7A gene may lead to infantile-onset cerebral degeneration (Menkes disease); occipital horn syndrome (OHS), a related but much milder illness; or an adult-onset isolated distal motor neuropathy. The ATP7A missense mutation T994I is located in the sixth transmembrane domain of ATP7A, represents one of the variants associated with the latter phenotype, and is associated with an abnormal interaction with p97/valosin-containing protein (VCP), a hexameric AAA ATPase (ATPase associated with diverse cellular activities) with multiple biological functions...
March 29, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29599191/ap-swath-reveals-direct-involvement-of-vcp-p97-in-integrated-stress-response-signaling-through-facilitating-crep-ppp1r15b-degradation
#17
Julia Hülsmann, Bojana Kravic, Matthias Weith, Matthias Gstaiger, Ruedi H Aebersold, Ben C Collins, Hemmo Meyer
The ubiquitin-directed AAA-ATPase VCP/p97 facilitates degradation of damaged or misfolded proteins in diverse cellular stress response pathways. Resolving the complexity of its interactions with partner and substrate proteins, and understanding its links to stress signaling is therefore a major challenge. Here, we used affinity-purification SWATH mass spectrometry (AP-SWATH) to identify proteins that specifically interact with the substrate-trapping mutant, p97-E578Q. AP-SWATH identified differential interactions over a large detection range from abundant p97 cofactors to pathway-specific partners and individual ligases such as RNF185 and MUL1 that were trapped in p97-E578Q complexes...
March 29, 2018: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/29588313/reliability-and-physiological-interpretation-of-pulmonary-gas-exchange-by-circulatory-equivalents-in-chronic-heart-failure
#18
Chunting Tan, Harry B Rossiter, Janos Porszasz, T Scott Bowen, Klaus K Witte, William W Stringer, Richard Casaburi, James E Hansen
BACKGROUND: Peak ratios of pulmonary gas-exchange to ventilation during exercise (V˙O2/V˙E and V˙CO2/V˙E, termed "circulatory equivalents") are sensitive to heart failure (HF) severity, likely reflecting low and/or poorly distributed pulmonary perfusion. We tested whether peak V˙O2/V˙E and V˙CO2/V˙E would: (1) distinguish HF patients from controls; (2) be independent of incremental exercise protocol; and (3) correlate with lactate threshold (LT) and ventilatory compensation point (VCP), respectively...
March 27, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29578490/frontotemporal-dementia-and-chorea-associated-with-a-compound-heterozygous-trem2-mutation
#19
Veronica Redaelli, Ettore Salsano, Lara Colleoni, Paola Corbetta, Giovanni Tringali, Angelo Del Sole, Giorgio Giaccone, Giacomina Rossi
Frontotemporal dementia (FTD) is clinically characterized by behavioral changes, language impairment, and executive dysfunction. FTD usually belongs to the frontotemporal lobar degeneration (FTLD) disease group, and its familial forms are dominantly inherited and linked to a group of genes relevant to frontal and temporal brain pathology, such as MAPT, GRN, C9ORF72, TARDBP, CHMP2B, VCP, and FUS. However, FTD can also be associated with different clinical or pathological phenotypes caused by mutations in other genes, whose heredity can be dominant or recessive...
March 23, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29556769/sequential-high-intensity-focused-ultrasound-hifu-ablation-in-the-treatment-of-benign-multinodular-goitre-an-observational-retrospective-study
#20
Brian H H Lang, Yu-Cho Woo, Keith Wan-Hang Chiu
OBJECTIVES: Assessing the efficacy and safety of sequential high-intensity focused ultrasound (HIFU) ablation in a multinodular goitre (MNG) by comparing them with single HIFU ablation. METHODS: One hundred and four (84.6%) patients underwent single ablation of a single nodule (group I), while 19 (15.4%) underwent sequential ablation of two relatively-dominant nodules in a MNG (group II). Extent of shrinkage per nodule [by volume reduction ratio (VRR)], pain scores (by 0-10 visual analogue scale) during and after ablation, and rate of vocal cord palsy (VCP), skin burn and nausea/vomiting were compared between the two groups...
March 19, 2018: European Radiology
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