نتایج جستجو برای: vertical coplanar vcp
تعداد نتایج: 99181 فیلتر نتایج به سال:
Using exome sequencing, we identified a p.R191Q amino acid change in the valosin-containing protein (VCP) gene in an Italian family with autosomal dominantly inherited amyotrophic lateral sclerosis (ALS). Mutations in VCP have previously been identified in families with Inclusion Body Myopathy, Paget disease, and Frontotemporal Dementia (IBMPFD). Screening of VCP in a cohort of 210 familial ALS...
Valosin Containing Protein (VCP) disease is an autosomal dominant multisystem proteinopathy caused by mutations in the VCP gene, and is primarily associated with progressive muscle weakness, including atrophy of the pelvic and shoulder girdle muscles. Currently, no treatments are available and cardiac and respiratory failures can lead to mortality at an early age. VCP is an AAA ATPase multifunc...
Sir, de Bot et al. report on a novel autosomal-dominant form of hereditary spastic paraplegia due to a valosin-containing protein (VCP) missense mutation. Mutations of the human VCP gene on chromosome 9p13.3 have previously been described to cause IBMPFD (inclusion body myopathy with early onset Paget’s disease of bone and frontotemporal dementia, OMIM #167320) (for current review on IBMPFD see...
BACKGROUND Previous intraoperative nerve monitoring (IONM) studies have demonstrated modest-to-no benefit and did not include a nationwide sample of hospitals representative of broad thyroidectomy practices. This national study was designed to compare vocal cord paralysis (VCP) rates between thyroidectomy with IONM and without monitoring (conventional). STUDY DESIGN We performed a retrospecti...
Geldanamycin and Velcade, new anticancer drugs with novel mechanisms of action, are currently undergoing extensive clinical trials. Geldanamycin interrupts Hsp90 chaperone activity and causes down-regulation of its many client proteins by the ubiquitin-proteasome pathway; Velcade is a specific proteasome inhibitor. Misfolded Hsp90 clients within the endoplasmic reticulum (ER) lumen are cleared ...
VCP (p97, cdc48) is a hexameric AAA ATPase involved in multiple cellular functions, including degradation of proteins by the ubiquitin-proteasome system (UPS). We examined the consequences of the reduction of VCP levels after RNAi of VCP. A new stringent method of microarray analysis demonstrated that only 4 transcripts were non-specifically affected by RNAi, while ~30 transcripts were affected...
OBJECTIVES This study aims to evaluate the demographic and clinical characteristics of patients with idiopathic and non-idiopathic vocal cord paralysis (VCP). PATIENTS AND METHODS This retrospective cohort was performed on data extracted from medical files of 92 consecutive patients (43 males, 49 females; median age 52.1±23.1 years; min. 1 - max. 87) with VCP diagnosed in the otorhinolaryngol...
Arginine/lysine-rich motifs typically function as targeting signals for the translocation of proteins to the nucleus. Here, we demonstrate that such a motif consisting of four basic amino acids in the polyglutamine protein ataxin-3 (Atx-3) serves as a recognition site for the interaction with the molecular chaperone VCP. Through this interaction, VCP modulates the fibrillogenesis of pathogenic ...
Vaccinia replication is complex, as it involves both producing new infectious particles as well as a myriad of proteins dedicated to combating the host’s immune response. Although these proteins are often called “nonessential”, they are frequently needed for the virus to achieve maximum virulence in vivo. Two of these proteins are the vaccinia complement control protein (VCP) and A56, the vacci...
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