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The Journal of Creating Value (JCV)is a Scopus-indexed, peer-reviewed journal that has been published since 2015. Over the last decade, JCVhas played a pivotal role in enhancing knowledge creation and sharing among academia and practitioners in its chosen field. This study is an attempt to map the contribution of the journal in terms of classifying impactful authors, current and evolving trends and themes, and set a direction for future researchers and practitioners. The review of the articles published was performed using bibliometric analysis and techniques. A total of 165 articles published during the years 2015 up to 2023 were part of the scope of the study using the Scopus database. Open-source visualization software VOSviewer was used for analysis. Performance analysis and science mapping techniques were employed, and the findings indicate five major themes/clusters. Thirty per cent of the publications are from the USA. Other contributing countries include India, Germany, the Netherlands and the UK. Overall, there are significant global contributions as well. Insightful trends and themes in the field of creating value are documented, which could be a useful resource for prospective researchers.

μ-Crystallin, encoded by the CRYMgene, binds the thyroid hormones, T3and T4. Because T3and T4are potent regulators of metabolism and gene expression, and CRYMlevels in human skeletal muscle can vary widely, we investigated the effects of overexpression of Crym. We generated transgenic mice, Crymtg, that expressed Crymprotein specifically in skeletal muscle at levels 2.6–147.5 fold higher than in controls. Muscular functions, Ca2+transients, contractile force, fatigue, running on treadmills or wheels, were not significantly altered, although T3levels in tibialis anterior (TA) muscle were elevated ~190-fold and serum T4was decreased 1.2-fold. Serum T3and thyroid stimulating hormone (TSH) levels were unaffected. Crymtransgenic mice studied in metabolic chambers showed a significant decrease in the respiratory exchange ratio (RER) corresponding to a 13.7% increase in fat utilization as an energy source compared to controls. Female but not male Crymtg mice gained weight more rapidly than controls when fed high fat or high simple carbohydrate diets. Although labeling for myosin heavy chains showed no fiber type differences in TA or soleus muscles, application of machine learning algorithms revealed small but significant morphological differences between Crymtg and control soleus fibers. RNA-seq and gene ontology enrichment analysis showed a significant shift towards genes associated with slower muscle function and its metabolic correlate, β-oxidation. Protein expression showed a similar shift, though with little overlap. Our study shows that μ-crystallin plays an important role in determining substrate utilization in mammalian muscle and that high levels of μ-crystallin are associated with a shift toward greater fat metabolism.

Objective: To report the rate of major soft tissue complications after cochlear implantation and to describe the use of the temporoparietal fascia (TPF) flap for such complications.Study Design: Retrospective case series.Setting: Tertiary care, University Hospital.Subjects and Methods: Chart review of all patients who underwent cochlear device implantation over a 5-year period to identify patients and to determine the rate of soft tissue complications. Five patients with major soft tissue complications underwent TPF flap with device salvage or explantation/reimplantation.Results: The rate of major skin complications was 6 out of 281 (2.1%) over 5 years, with 5 patients undergoing TPF flap. The average follow-up was 25.8 months (range, 5-58 months). TPF flap represented the definitive, successful solution for all 5 patients. One postoperative hematoma occurred after TPF flap, with no long-term sequelae. The average hospital length of stay was 2.2 nights (range, 1-5 nights). One patient required IV antibiotics for 4 weeks; the remaining patients were treated with a postoperative course of oral antibiotics. The original device remained in place for 4 patients, while one case required device explantation and staged re-implantation. Post-TPF flap hearing results were equal to if not superior to their preoperative results.Conclusion: Major soft tissue complications following cochlear device implantation are rare. The temporoparietal fascia flap is an excellent option for reconstruction of device site soft tissue dehiscences when local wound care and primary closure are not sufficient, and can potentially prevent explantation of a functional implant.

Mutations in the dysferlin gene (DYSF) lead to human muscular dystrophies known as dysferlinopathies. The dysferlin-deficient A/J mouse develops a mild myopathy after 6 months of age, and when younger models the subclinical phase of the human disease. We subjected the tibialis anterior muscle of 3- to 4-month-old A/J mice to in vivolarge-strain injury (LSI) from lengthening contractions and studied the progression of torque loss, myofiber damage, and inflammation afterward. We report that myofiber damage in A/J mice occurs before inflammatory cell infiltration. Peak edema and inflammation, monitored by magnetic resonance imaging and by immunofluorescence labeling of neutrophils and macrophages, respectively, develop 24 to 72 hours after LSI, well after the appearance of damaged myofibers. Cytokine profiles 72 hours after injury are consistent with extensive macrophage infiltration. Dysferlin-sufficient A/WySnJ mice show much less myofiber damage and inflammation and lesser cytokine levels after LSI than do A/J mice. Partial suppression of macrophage infiltration by systemic administration of clodronate-incorporated liposomes fails to suppress LSI-induced damage or to accelerate torque recovery in A/J mice. The findings from our studies suggest that, although macrophage infiltration is prominent in dysferlin-deficient A/J muscle after LSI, it is the consequence and not the cause of progressive myofiber damage.

Mutations in the DYSF gene that severely reduce the levels of the protein dysferlin are implicated in muscle-wasting syndromes known as dysferlinopathies. Although studies of its function in skeletal muscle have focused on its potential role in repairing the plasma membrane, dysferlin has also been found, albeit inconsistently, in the sarcoplasm of muscle fibers. The aim of this article is to study the localization of dysferlin in skeletal muscle through optimized immunolabeling methods. We studied the localization of dysferlin in control rat skeletal muscle using several different methods of tissue collection and subsequent immunolabeling. We then applied our optimized immunolabeling methods on human cadaveric muscle, control and dystrophic human muscle biopsies, and control and dysferlin-deficient mouse muscle. Our data suggest that dysferlin is present in a reticulum of the sarcoplasm, similar but not identical to those containing the dihydropyridine receptors and distinct from the distribution of the sarcolemmal protein dystrophin. Our data illustrate the importance of tissue fixation and antigen unmasking for proper immunolocalization of dysferlin. They suggest that dysferlin has an important function in the internal membrane systems of skeletal muscle, involved in calcium homeostasis and excitation-contraction coupling.

Mutations in the gene DYSF, which codes for the protein dysferlin, underlie Miyoshi myopathy and limb-girdle muscular dystrophy 2B in humans and produce a slowly progressing skeletal muscle degenerative disease in mice. Dysferlin is a Ca2+-sensing, regulatory protein that is involved in membrane repair after injury. To assess the function of dysferlin in healthy and dystrophic skeletal muscle, we generated skeletal muscle–specific transgenic mice with threefold overexpression of this protein. These mice were phenotypically indistinguishable from wild-type, and more importantly, the transgene completely rescued the muscular dystrophy (MD) disease in Dysf-null A/Jmice. The dysferlin transgene rescued all histopathology and macrophage infiltration in skeletal muscle of Dysf−/−A/Jmice, as well as promoted the rapid recovery of muscle function after forced lengthening contractions. These results indicate that MD in A/Jmice is autonomous to skeletal muscle and not initiated by any other cell type. However, overexpression of dysferlin did not improve dystrophic symptoms or membrane instability in the dystrophin-glycoprotein complex–lacking Scgd(δ-sarcoglycan) null mouse, indicating that dysferlin functionality is not a limiting factor underlying membrane repair in other models of MD. In summary, the restoration of dysferlin in skeletal muscle fibers is sufficient to rescue the MD in Dysf-deficient mice, although its mild overexpression does not appear to functionally enhance membrane repair in other models of MD.

Obscurin is a large (∼800-kDa), modular protein of striated muscle that concentrates around the M-bands and Z-disks of each sarcomere, where it is well positioned to sense contractile activity. Obscurin contains several signaling domains, including a rho-guanine nucleotide exchange factor (rhoGEF) domain and tandem pleckstrin homology domain, consistent with a role in rho signaling in muscle. We investigated the ability of obscurin's rhoGEF domain to interact with and activate small GTPases. Using a combination of in vitro and in vivo approaches, we found that the rhoGEF domain of obscurin binds selectively to rhoA, and that rhoA colocalizes with obscurin at the M-band in skeletal muscle. Other small GTPases, including rac1 and cdc42, neither associate with the rhoGEF domain of obscurin nor concentrate at the level of the M-bands. Furthermore, overexpression of the rhoGEF domain of obscurin in adult skeletal muscle selectively increases rhoA expression and activity in this tissue. Overexpression of obscurin's rhoGEF domain and its effects on rhoA alter the expression of rho kinase and citron kinase, both of which can be activated by rhoA in other tissues. Injuries to rodent hindlimb muscles caused by large-strain lengthening contractions increases rhoA activity and displaces it from the M-bands to Z-disks, similar to the effects of overexpression of obscurin's rhoGEF domain. Our results suggest that obscurin's rhoGEF domain signals at least in part by inducing rhoA expression and activation, and altering the expression of downstream kinases in vitro and in vivo.

The protein, dysferlin, mediates sarcolemmal repair in vitro, implicating defective membrane repair in dysferlinopathies. To study the role of dysferlin in vivo, we assessed contractile function, sarcolemmal integrity, and myogenesis before and after injury from large-strain lengthening contractions in dysferlin-null and control mice. We report that dysferlin-null muscles produce higher contractile torque, and are equally susceptible to initial injury but recover from injury more slowly. Two weeks after injury, control muscles retain fluorescein dextran and do not show myogenesis. Dysferlin-null muscles do not retain fluorescein dextran, and show necrosis followed by myogenesis. Our data indicate that recovery of control muscles from injury primarily involves sarcolemmal repair whereas recovery of dysferlin-null muscles primarily involves myogenesis without repair and long-term survival of myofibers.

Objective: To investigate the results of cochlear implantation in subjects with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas (VS).Study Design: Retrospective case series.Setting: University-based tertiary referral center.Subjects: Five subjects with NF2 and severe-to-profound sensorineural hearing loss.Intervention: Cochlear implantation.Main Outcome Measure: Surgical outcomes and audiometric performance after cochlear implantation.Results: Five subjects (3 female, 2 male) were included in the study. The mean age at the time of implantation was 54 years old (range 35-78 years). Follow-up after cochlear implantation averaged 38 months (range 21-106 months). In the 5 implanted ears, 2 had no prior treatment, 1 had undergone prior radiation therapy, 1 underwent prior microsurgical removal, and 1 underwent prior microsurgical removal with adjuvant radiation therapy. The mean ipsilateral VS dimensions at time of implantation were 14 mm × 7.2 mm × 6.1 mm (mediolateral × anteroposterior × craniocaudal). Following cochlear implant activation, all 5 subjects achieved sound awareness, open set speech recognition, and 4 continue to be daily users of the devices.Conclusion: Cochlear implantation is a viable hearing rehabilitation option for subjects with NF2 and severe-to-profound sensorineural hearing loss. All subjects reported benefit with their cochlear implant, including open set speech recognition, enhanced lip-reading skills and environmental awareness of sound. Four subjects continued to demonstrate improved open-set speech recognition at the time of their last evaluations.

Reports on the use of a multiplex (MUX) blowout preventer (BOP) system that provides quicker riser decoupling and speeds shear and pipe ram closing for deep and ultra-deep water petroleum operations. MUX advantages; Deepwater control systems; Engineered components; Additional drillship features.