Inherited Form Research Articles

The art of failure in Jean Rhys's Voyage in the Dark and Una Marson's The Moth and the Star

ABSTRACT Though both writers migrated from the Caribbean and lived at the same time in England in the 1930s, Jean Rhys (1890–1979) and Una Marson (1905–1965) did not run in the same circles – or know of each other. Marson's poetry and plays emerge from her anticolonial, feminist political commitments while Rhys has often been read as an isolated figure in modernist and Caribbean literary histories. They each had a complicated relationship to both European modernism and to mid-twentieth-century Anglophone Caribbean literature. Rhys and Marson have been put in implicit conversation in the wake of the new modernist studies because they are uneasily positioned within familiar canons of twentieth-century literature. Importantly, as this article argues, both writers also focus on failure – personal, aesthetic, and political – in their key works exploring migration in the 1930s – Rhys's novel Voyage in the Dark (1934) and Marson's poetry collection The Moth and the Star (1937). Failure in these texts is double sided, recasting personal and aesthetic failure as the failure of dominant narratives to recognize their characters. Reading these texts together, this article argues that late colonial women writers at once registered and challenged dominant conceptions of failure in their reconstruction of inherited forms.

Impact of Resolvin-D1 in the resolution of atrial inflammation and arrhythmogenic cardiac remodelling in a rat model of myosin light chain 4 (MYL-4) mutation

Atrial fibrillation (AF) is the most common heart rhythm disorder. AF risk factors include age, hypertension, obesity, or myocardial infarction. AF can cause severe complications such as stroke and sudden death. Hence, AF is a major public health issue. Proteins called actins and myosins interact to allow myocardial contractions. A mutation affecting the gene encoding a myosin light chain type 4 (MYL-4) protein is associated with a genetically inheritable form of AF. Furthermore, we have recently demonstrated that MYL-4 rats develop atrial inflammation and fibrosis. Conventional anti-inflammatory medications are ineffective against AF. Bioactive endogenous autacoids, called specialized proresolving mediators, including resolvin-D1: RvD1, have been described to promoting inflammation-resolution and potentially reduce AF occurrence. To elucidate the impact of a triggered resolution of inflammation in the prevention of AF. Resolution of atrial inflammation by daily treatment with RvD1 prevents the development of AF in a rat model of MYL-4 mutation. We have developed a colony of male and female Sprague-Dawley Knock-In rats, mutated on the Myl-4 gene. A dose of 2 μg/kg/d RvD1 (or equivalent dose of vehicle) was administered to wild-type (WT) and MYL-4 rats for 21 days. AF was assessed by in vivo electrophysiological study. The structure of the atria was studied by echocardiography, histology and electron microscopy. The expression levels of proteins (by western blotting) and genes (by qPCR) involved in inflammation and cellular apoptosis have been assessed in the right and left atria of MYL-4 rats compared to WT. Compared to WT rat, MYL-4 mutation causes an increased risk of AF, associated with an inflammatory and apoptotic profile in the atria. We observed that daily treatment with RvD1 prevents atrial inflammation (IL-1β), reduces cell death (Bax, Bcl2) and attenuates atrial structural remodelling (dilation, hypertrophy). Inflammation plays a crucial role in the development of arrhythmogenic atrial remodeling observed in rats carrying MYL4 mutation. In addition, this project contributes to highlight the potential therapeutic effects of the RvD1 treatment, to resolve inflammation and prevent AF in the context of a rhythm disturbances provoked by cardiac mutations.

FMRP phosphorylation and interactions with Cdh1 regulate association with dendritic RNA granules and MEF2-triggered synapse elimination

Fragile X Messenger Ribonucleoprotein (FMRP) is necessary for experience-dependent, developmental synapse elimination and the loss of this process may underlie the excess dendritic spines and hyperconnectivity of cortical neurons in Fragile X Syndrome, a common inherited form of intellectual disability and autism. Little is known of the signaling pathways that regulate synapse elimination and if or how FMRP is regulated during this process. We have characterized a model of synapse elimination in CA1 neurons of organotypic hippocampal slice cultures that is induced by expression of the active transcription factor Myocyte Enhancer Factor 2 (MEF2) and relies on postsynaptic FMRP. MEF2-induced synapse elimination is deficient in Fmr1 KO CA1 neurons, and is rescued by acute (24 h), postsynaptic and cell autonomous reexpression of FMRP in CA1 neurons. FMRP is an RNA binding protein that suppresses mRNA translation. Derepression is induced by posttranslational mechanisms downstream of metabotropic glutamate receptor signaling. Dephosphorylation of FMRP at S499 triggers ubiquitination and degradation of FMRP which then relieves translation suppression and promotes synthesis of proteins encoded by target mRNAs. Whether this mechanism functions in synapse elimination is not known. Here we demonstrate that phosphorylation and dephosphorylation of FMRP at S499 are both necessary for synapse elimination as well as interaction of FMRP with its E3 ligase for FMRP, APC/Cdh1. Using a bimolecular ubiquitin-mediated fluorescence complementation (UbFC) assay, we demonstrate that MEF2 promotes ubiquitination of FMRP in CA1 neurons that relies on activity and interaction with APC/Cdh1. Our results suggest a model where MEF2 regulates posttranslational modifications of FMRP via APC/Cdh1 to regulate translation of proteins necessary for synapse elimination.

OPTN: A Possible Genetic Cause of Both CBS and ALS (P9-11.013)

<h3>Objective:</h3> To report a patient with a mutation typically associated with familial amyotrophic lateral sclerosis (ALS), but instead presenting with the corticobasal syndrome (CBS) phenotype. <h3>Background:</h3> CBS is defined by atypical parkinsonism and specific examination findings indicating cortical dysfunction. In contrast, corticobasal degeneration (CBD) is a pathological entity and is the principal pathology underlying CBS. CBD and frontotemporal dementia (FTD) often involve tau deposition, but in different topographical distributions, and also in different molecular forms. Many cases of CBD and FTD may, in addition, overlap clinically. By comparison, there is significantly less clinical overlap between ALS and the pathologies that cause CBS. Here we report a case of CBS linked to a mutation in the OPTN gene. This individual has several close relatives diagnosed with familial ALS who also have been found to carry the same OPTN mutation. <h3>Design/Methods:</h3> NA <h3>Results:</h3> We report a 49-year-old man with 6 months of involuntary movements of the right upper extremity, followed by gait unsteadiness and memory changes. Family history was notable for two siblings with ALS linked to a mutation in the OPTN gene. On exam, we noted right hemidystonia, as well as bradykinesia and brisk reflexes in the right arm. There was gait ataxia, as well as right-sided astereognosis, agraphesthesia, tactile extinction, and ideomotor apraxia. No ocular abnormalities, orthostasis, or other upper-motor neuron features were seen. MRI showed left peri-Rolandic fissure atrophy. Gene testing revealed homozygosity for a loss of function splice-site mutation in the OPTN gene. <h3>Conclusions:</h3> This case illustrates differential effects of the same gene mutation in different family members. It is interesting that the pathology causing CBS in this patient shares the same mutation with an inherited form of ALS. The heterogeneity of OPTN phenotypes may be a consequence of the same neuropathology, but with differential topographical distribution in the nervous system. <b>Disclosure:</b> Dr. Feldman has nothing to disclose. Dr. Benatar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. The institution of Dr. Benatar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arrowhead. Dr. Benatar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Annexon. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UniQure. Dr. Benatar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunovant. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alector. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Dr. Benatar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Benatar has received intellectual property interests from a discovery or technology relating to health care. Dr. Benatar has received intellectual property interests from a discovery or technology relating to health care. Dr. Saraf Lavi has nothing to disclose. Dr. Latchman has nothing to disclose. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amneal. Dr. Singer has received personal compensation in the range of $10,000-$49,999 for serving as a Video Panel with CNS Ratings LLC. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Course Organiztion with Omniprex Scientific Medical Eduaction. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Reviewer with Health Monitor Network. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Movement Disorders Society. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Workshop Moderator with International Neurotoxin Association Inc. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Lecturer with CME Outfitteres LLC.

How Small-scale Fishermen Survive During the Pandemic of Covid-19

&#x0D; &#x0D; &#x0D; &#x0D; This study aims to examine how the Kendari Bay’s coastal fishermen survive during the Covid-19 pandemic, especially from an economic perspective. However, the Covid-19 pandemic that occurred in the last three years has been able to shake the economy in several levels of society, including small-scale fishermen. The object of this research is 50 small-scale fishermen on the coast of Kendari Bay with data collection methods using surveys and interviews. Data analysis was carried out to reveal the characteristics of small-scale fishermen on the coast of Kendari Bay, to examine the impact of the COVID-19 pandemic on fishermen's income, and the constraints faced by fishermen from an economic perspective. The results show that small-scale fishermen averagely work as fishermen from a young age, as a form of inheritance from their parents and cannot complete basic compulsory education. Surprisingly, the Covid-19 pandemic does not have much impact on fishermen's income because the biggest impact comes from weather factors and the high cost of boat fuel. One of many issues that the fishermen need to deal is the difficulty on accessing capital and financial to start a business. This is happening due to limited information for these fishermen. The novelty of this research is in the study of the impact of the pandemic which is associated with access to public finance.&#x0D; &#x0D; &#x0D; &#x0D;

CRISPR-mediated optogene expression from a cell-specific endogenous promoter in retinal ON-bipolar cells to restore vision

Retinitis pigmentosa, an inherited form of retinal degeneration, is characterized by a progressive loss of rods and subsequent degeneration of cones, leading to blindness. However, the remaining neural portion of the retina (bipolar and ganglion cells) remains anatomically and functionally intact for an extended time. A possible treatment to restore the light sensitivity of the retina consists of rendering the remaining retinal cells photosensitive using optogenetic tools like, for example, Opto-mGluR6, a light-sensitive mGluR6 receptor. We have previously demonstrated that AAV vector-mediated expression of Opto-mGluR6 in ON-bipolar cells restores visual function in otherwise blind mice. However, classical gene supplementation therapy still suffers from high off-target expression rates and uncontrollable target gene expression levels that may lead to either cytotoxicity or lack of functional restoration. To address these issues and achieve cell-specific and endogenously controlled Opto-mGluR6 expression, we employed the CRISPR/Cas technology—in particular, homology-independent targeted integration (HITI) and microhomology-dependent targeted integration (MITI)—to knock-in the Opto-mGluR6 gene behind the ON-bipolar cell-specific GRM6 promoter. We compared four Cas systems in vitro and show that SpCas9 for HITI and LbCpf1 for MITI are well suited to promoting knock-in. As AAV2-mediated ON-bipolar cell transduction resulted in inefficiency, we evaluated Exo-AAVs as delivery vehicles and found Exo-AAV1 efficient for targeting ON-bipolar cells. We demonstrate that intravitreal injection of Exo-AAV1 carrying vectors that promote MITI significantly improved visual acuity in otherwise blind rd1 mice. We conclude by confirming and providing a qualitative evaluation of the MITI-mediated knock-in in the correct genomic locus.

Combined Alport syndrome, Klinefelter syndrome and Fanconi syndrome in a Chinese boy.

Alport syndrome (AS) is a progressive renal disease characterized by hematuria and progressive renal failure. X-linked dominant (XLAS) is the major inheritance form, accounting for almost 80% of the cases, caused by mutations in COL4A5 genes. Klinefelter syndrome (KS) is the most common genetic cause of human male gonadal dysgenesis. AS and KS are both rare disease, there are only three cases of combined AS and KS in the literatures. Fanconi syndrome (FS) caused by AS is also very rare. We report here the first case combined AS, KS and FS in a Chinese boy. We suggest that the severe renal phenotype and FS might be due to the two homozygous COL4A5 variants in our boy, and cases of AS combined KS will be good research objects for X chromosome inactivation.

Цифровое наследство

Introduction. The study deals with the problematic issues of digital inheritance when studying some aspects of electronic money, the accounts of the testator, and other digital assets. The purpose of the study is to show the social significance and differentiation of the last will of the legislator and public interest in the results of creative activity, including those existing in the form of digital inheritance, and to identify the legal consequences of the relationship between heirs and society, the last will of the testator. Methods. The methods of legal formalism, synthesis, and analysis are used. Results. The author’s position, substantiated in the work, is based on domestic and foreign legislation and the judicial practice of its application. Conclusions. The recommendations are given, the proposals and directions for eliminating legal conflicts are formulated, and specific measures are proposed to improve the legislation in the field of inheritance of the testator’s digital assets in addition to a theoretical understanding of the category under consideration.

Galbut Virus Infection Minimally Influences Drosophila melanogaster Fitness Traits in a Strain and Sex-Dependent Manner.

Galbut virus (family Partitiviridae) infects Drosophila melanogaster and can be transmitted vertically from infected mothers or infected fathers with near perfect efficiency. This form of super-Mendelian inheritance should drive infection to 100% prevalence, and indeed, galbut virus is ubiquitous in wild D. melanogaster populations. However, on average, only about 60% of individual flies are infected. One possible explanation for this is that a subset of flies are resistant to infection. Although galbut virus-infected flies appear healthy, infection may be sufficiently costly to drive selection for resistant hosts, thereby decreasing overall prevalence. To test this hypothesis, we quantified a variety of fitness-related traits in galbut virus-infected flies from two lines from the Drosophila Genetic Reference Panel (DGRP). Galbut virus-infected flies had no difference in average lifespan and total offspring production compared to their uninfected counterparts. Galbut virus-infected DGRP-517 flies pupated and eclosed faster than their uninfected counterparts. Some galbut virus-infected flies exhibited altered sensitivity to viral, bacterial, and fungal pathogens. The microbiome composition of flies was not measurably perturbed by galbut virus infection. Differences in phenotype attributable to galbut virus infection varied as a function of fly sex and DGRP strain, and differences attributable to infection status were dwarfed by larger differences attributable to strain and sex. Thus, galbut virus infection does produce measurable phenotypic changes, with changes being minor, offsetting, and possibly net-negative.

Peranan Pemuda dalam Melesarikan Tradisi Lampu Colok di Desa Pangkalan Batang Kecamatan Bengkalis

This study aims to describe the role of youth in preserving the plug-in lamp tradition in Pangkalan Batang village, Bengkalis sub-district. This research method is descriptive research with a qualitative approach. In this study, researchers conducted research in Pangkalan Batang Village, Bengkalis District. Data collection techniques in this study are observation, interviews, and documentation. The informants in this study consisted of 7 (seven) people, namely the Chairperson of the Bengkalis Regency LAMR Customary Density Council, the Bengkalis Regency Sports and Culture Tourism Office, traditional leaders and the head of the hamlet of Pangkalan Batang village, the chairman of the Karang Taruna and the head of the Pangkalan Batang village plug. The data analysis technique follows the flow model proposed by Miles and Huberman, namely data reduction, data presentation, conclusion and verification. The results in this study explain that in the lamp plug tradition there is a role for youth in the form of protection, development, utilization, and inheritance.

The Ubiquitin Proteasome System as a Therapeutic Area in Parkinson's Disease.

Parkinson's disease (PD) is the most common neurodegenerative movement disorder. There are no available therapeutics that slow or halt the progressive loss of dopamine-producing neurons, which underlies the primary clinical symptoms. Currently approved PD drugs can provide symptomatic relief by increasing brain dopamine content or activity; however, the alleviation is temporary, and the effectiveness diminishes with the inevitable progression of neurodegeneration. Discovery and development of disease-modifying neuroprotective therapies has been hampered by insufficient understanding of the root cause of PD-related neurodegeneration. The etiology of PD involves a combination of genetic and environmental factors. Although a single cause has yet to emerge, genetic, cell biological and neuropathological evidence implicates mitochondrial dysfunction and protein aggregation. Postmortem PD brains show pathognomonic Lewy body intraneuronal inclusions composed of aggregated α-synuclein, indicative of failure to degrade misfolded protein. Mutations in the genes that code for α-synuclein, as well as the E3 ubiquitin ligase Parkin, cause rare inherited forms of PD. While many ubiquitin ligases label proteins with ubiquitin chains to mark proteins for degradation by the proteasome, Parkin has been shown to mark dysfunctional mitochondria for degradation by mitophagy. The ubiquitin proteasome system participates in several aspects of the cell's response to mitochondrial damage, affording numerous therapeutic opportunities to augment mitophagy and potentially stop PD progression. This review examines the role and therapeutic potential of such UPS modulators, exemplified by both ubiquitinating and deubiquitinating enzymes.

Novel Candidate loci and Pathogenic Germline Variants Involved in Familial Hematological Malignancies Revealed by Whole-Exome Sequencing.

The familial occurrence of hematological malignancies has been underappreciated. Recent studies suggest that up to 15% of adults with myeloid neoplasms carry germline pathogenic variants in cancer-predisposing genes. This study aimed to identify the underlying germline predisposition variant in patients with a strong family or personal onco-hematological history using whole exome sequencing on sixteen uncharacterized individuals. It was carried out in two groups of patients, one with samples available from two affected relatives (Cohort A) and one with available samples from the index case (Cohort B). In Cohort A, six families were characterized. Two families shared variants in genes associated with DNA damage response and involved in cancer development (CHEK2 and RAD54L). Pathogenic or likely pathogenic germline variants were also found in novel candidate genes (NFATC2 and TC2N). In two families, any relevant pathogenic or likely pathogenic genomic variants were identified. In Cohort B, four additional index cases were analyzed. Three of them harbor clinically relevant variants in genes with a probable role in the development of inherited forms of hematological malignancies (GATA1, MSH4 and PRF1). Overall, whole exome sequencing is a useful approach to achieve a further characterization of these patients and their mutational spectra. Moreover, further investigations may help improve optimization for disease management of affected patients and their families.

Sporadic Spinal-Onset Amyotrophic Lateral Sclerosis Associated with Myopathy in Three Unrelated Portuguese Patients

Amyotrophic lateral sclerosis (ALS) and myopathy have been already described as part of a common genetic syndrome called multisystem proteinopathy. They may occur together or not, and can be associated with other clinical features such as frontotemporal dementia and Paget's bone disease. In addition, primary skeletal muscle involvement has been also reported in inherited forms of lower motor neuron disease, in spinal-bulbar muscular atrophy and in spinal muscular atrophy. We aim to characterize three sporadic, spinal-onset ALS patients, one with a concurrent non-specific myopathy, and two with a previous diagnosis of myopathy before upper and lower motor neuron signs emerged. Perhaps our sporadic ALS cases associated with myopathy share a common, but still unknown, pathogenic background. These cases raise the paradigm of a possible interplay between skeletal muscle degeneration and motor neuron damage.

MRAP2 regulates energy homeostasis by promoting primary cilia localization of MC4R.

The G protein-coupled receptor melanocortin-4 receptor (MC4R) and its associated protein melanocortin receptor-associated protein 2 (MRAP2) are essential for the regulation of food intake and body weight in humans. MC4R localizes and functions at the neuronal primary cilium, a microtubule-based organelle that senses and relays extracellular signals. Here, we demonstrate that MRAP2 is critical for the weight-regulating function of MC4R neurons and the ciliary localization of MC4R. More generally, our study also reveals that GPCR localization to primary cilia can require specific accessory proteins that may not be present in heterologous cell culture systems. Our findings further demonstrate that targeting of MC4R to neuronal primary cilia is essential for the control of long-term energy homeostasis and suggest that genetic disruption of MC4R ciliary localization may frequently underlie inherited forms of obesity.

Early 7,8-Dihydroxyflavone Administration Ameliorates Synaptic and Behavioral Deficits in the Young FXS Animal Model by Acting on BDNF-TrkB Pathway.

Fragile X syndrome (FXS) is the leading inherited form of intellectual disability and the most common cause of autism spectrum disorders. FXS patients exhibit severe syndromic features and behavioral alterations, including anxiety, hyperactivity, impulsivity, and aggression, in addition to cognitive impairment and seizures. At present, there are no effective treatments or cures for FXS. Previously, we have found the divergence of BDNF-TrkB signaling trajectories is associated with spine defects in early postnatal developmental stages of Fmr1 KO mice. Here, young fragile X mice were intraperitoneal injection with 7,8-Dihydroxyflavone (7,8-DHF), a high affinity tropomyosin receptor kinase B (TrkB) agonist. 7,8-DHF ameliorated morphological abnormities in dendritic spine and synaptic structure and rescued synaptic and hippocampus-dependent cognitive dysfunction. These observed improvements of 7,8-DHF involved decreased protein levels of BDNF, p-TrkBY816, p-PLCγ, and p-CaMKII in the hippocampus. In addition, 7,8-DHF intervention in primary hippocampal neurons increased p-TrkBY816 and activated the PLCγ1-CaMKII signaling pathway, leading to improvement of neuronal morphology. This study is the first to account for early life synaptic impairments, neuronal morphological, and cognitive delays in FXS in response to the abnormal BDNF-TrkB pathway. Present studies provide novel evidences about the effective early intervention in FXS mice at developmental stages and a strategy to produce powerful impacts on neural development, synaptic plasticity, and behaviors.

Familial Reactive Perforating Collagenosis: Clinical, Dermoscopy, and Histopathological Features in Two Siblings

Transepidermal elimination disorders include reactive perforating collagenosis (RPC), perforating folliculitis, elastosis perforans serpiginosa, and Kyrle’s disease. RPC is a rare transepidermal elimination disorder wherein altered collagen is extruded through the epidermis. It has two types: rare inherited form and relatively common acquired form. Most inherited cases present during infancy or early childhood with papules on extensor surface of hands, elbows, knees preceded by superficial trauma. We report two female siblings of 11 and 10 years with multiple, symmetrically distributed, hyperpigmented papules with central keratotic plug, predominantly over extensor aspects of extremities with no aggravating and relieving factors. Clinical diagnosis of familial RPC was confirmed by consistent dermoscopic and histopathological findings. Both children were started on oral isotretinoin 10 mg/day with topical 12% ammonium lactate.

Neo-classical “Structural Functionalism” on the “Traditional-Modern” Transformation of Literature and Art Cultural Heritage

As inherited experience and wisdom from traditional culture, cultural heritage is an important part of modern social life. Using the Neo-classical “Structural Functionalism”, this paper analyzes the structural and functional changes in Literature and Art Cultural Heritage (LACH)1 in the transition from tradition to modern times. In a modern society, there are multiple inheritance forms of LACH, such as Urban Revitalization, Characteristic Towns, cultural and tourism-related performing arts, festival activities and temple fairs, etc. They exhibit diverse values with all kinds of structure-function, such as embodied in original, associated or fragmented forms.

Loss of surface transport is a main cellular pathomechanism of CRB2 variants causing podocytopathies.

Crumbs2 (CRB2) is a central component of the renal filtration barrier and part of the slit diaphragm, a unique cell contact formed by glomerular podocytes. Some CRB2 variants cause recessive inherited forms of steroid-resistant nephrotic syndrome. However, the disease-causing potential of numerous CRB2 variants remains unknown. Here, we report the establishment of a live-cell imaging-based assay, allowing a quantitative evaluation of the pathogenic potential of so far non-categorized CRB2 variants. Based on in silico data analysis and protein prediction software, putative disease-associated CRB2 missense variants were selected, expressed as CRB2-GFP fusion proteins, and analyzed in reporter cell lines with BFP-labeled plasma membrane. We found that in comparison with PM-localized WT, disease-associated CRB2 variants remained predominantly at the ER. Accumulation at the ER was also present for several non-characterized CRB2 variants and variants in which putative disulfide bridge-forming cysteines were replaced. Strikingly, WT CRB2 retained inside the ER in cells lacking protein disulfide isomerase A3, indicating that posttranslational modification, especially the formation of disulfide bridges, is a crucial step for the CRB2 PM transport.

Innovative Reflections on the Visual Language of Batik in the Bouyei Ethnic Group of Guizhou, China

Intangible cultural heritage should not only be inherited and protected but also developed and innovated. Only in this way can traditional cultural heritage keep pace with the times and fully demonstrate its value and significance, and the Guizhou Bouyei batik is no exception. Batik is one of China's ancient folk traditional printing and dyeing processes. It is a common dyeing process and an intangible cultural heritage of ancient China. The art of batik is a time-tested craft. The technique of batik has more than one hundred years, during which it has become more and more complicated and improved. Guizhou is known as the birthplace of batik. Batik is an important symbol of Guizhou that can be traced back to the Qin and Han Dynasties. Guizhou Province is the main population center of the Bouyei ethnic group in China. The visual language is the overall display of the artistic design effect, and the Guizhou Bouyei batik has artistic attributes. Through the innovation of batik materials, technical methods, forms of expression and inheritance, the innovation of visual language will eventually lead to the formation of a new look of batik of Bouyei Nationality in Guizhou.

Patterns and Programs: Replication and Creativity in the Place-Based Knitting of Shetland and Ireland

Styles of knitwear associated with places, such as Shetland’s “fair isle” and Ireland’s “aran” knitting, are viewed through the lens of “tradition” by many consumers and makers. The creative process which results in such textile products is widely recognized as a communal one and set within centuries-long (real and imagined) historical contexts. This popular understanding includes a valorization of hand skills, particularly the improvizational fluency of expert hand knitters of the past. Their remembered ability to work and re-work inherited forms into innovative objects with minimal or no written instruction is an example of the “improvizational dynamics” which make “copying and reproduction…part and parcel of the creative process”. During the twentieth century, Shetland “fair isle” and Irish “aran” knitting experienced commercial success fueled by both hand and machine making practices, part of their industrial heritage which influences production today. With the increasing globalization of the mass market apparel industry, “fair isle” and “aran” knitwear styles have been absorbed into an international design lexicon that is drawn on by a wide range of producers. Contemporary commercial producers of knitwear in Shetland and Ireland distinguish themselves from their international mass market counterparts by emphasizing their enmeshment in a living, longstanding, communal creative web of hand and machine-making practices. This article explores the relationship between creativity and repetition in contemporary Shetland “fair isle” and Irish “aran” knitting, drawing on interviews and observations from two studies, one about “skill” in the knit industry of Shetland and Ireland funded by the Carnegie Trust (2018–19) and one about the value of Shetland hand knitting (2016–17). The article argues that repetition (including that enabled by “traditional” hand knitting patterns and the designs programmed into automated production systems) and the mutually disruptive interplay of hand and machine processes are key to creativity in place-based knitting practices.