The Community Influence of Sponge and Coral Aquaculture in Zanzibar

Aquaculture has been presented as a means of income for coastal communities, particularly in the context of climate change and resource exploitation. The NGO Marine Cultures in Jambiani, Zanzibar has established a sponge cultivation program for women in response to declining feasibility of seaweed farming from warming ocean temperatures. In addition, the organization strives to restore a severely damaged reef while providing employment for coral farmers and tour boat operators. This study analyzed the influence of aquaculture on community stakeholders, primarily with respect to sponge cultivation and secondarily in regard to coral farms. Using Marine Cultures as a case study, the principal aim was to investigate the impacts of sponge farms on the lives of women, with supplementary examination of the coral project and potential for community benefit. Participant observation and interviews were employed to generate qualitative data about the farms themselves, Marine Cultures, and the individuals impacted, predominantly women sponge farmers. The results of the study were a holistic narrative of Marine Cultures, four biographical sketches (three sponge farmers and one coral farmer) and a clear representation of aquaculture’s benefits to individuals. Dhahania Kilimo bahari kilifanywa ikiwa ni njia ya kujipatia kipato kwa jamii ya watu wa mwambao wa Jambiani, Katika kukabiliana na hali ya tabia nchi na uvunaji wa maliasili. Taasisi isiyo ya kiserikali ya Kilimo bahari katika eneo la Jambiani, Zanzibar walianzisha upandaji wa spongi bahari (vinja bahari) kwa Wanawake kutoka na wasiwasi wa kushuka kwa uzalishaji wa mwani kutokana na kupanda kwa joto la bahari. Kwa kuongezeka, taasisi hii inaangalia uwezekano wa kurejesha matumbawe yaliyoathiriwa kwa kiwango kikubwa wakati huohuo wakitoa ajira kwa Wakulima wa Matumbawe pamoja waendesha boti. Utafiti huu ulichunguza pamoja na kuchambua ushawishi wa kilimo bahari kwa washika dau wa jamii ya Jambiani, kimsingi kwa kuzingatia kilimo cha Spongi na kilimo cha Matumbawe. Kwa kutumia jumuiya ya “Marine Culture” eneo la Kujifunzia, Madhumuni ya msingi yalikuwa ni utafiti kilimo cha mashamba ya spongi pamoja na maisha ya wakulima wa kike, utafiti wa ziada wa kilimo cha Matumbawe na faida kilimo kwa wanajamii. Uchunguzi kwa vitendo na mahojiano ulifanyika ili kuweza kupata data kuhusiana na mashamba yao, Kilimo bahari, na waathirika, Mara nyingi wakulima wa spongi ni wanawake. Matokeo ya utafiti huu ni kwa ujumla yanasimulia “Marine Cultures,” 4 michoro ya kibinadamu (3 wakulima wa spongi, 1 mkulima wa matumbawe) na uwakilishi mzuri wa faida kwa kilimo mmoja

Pathogenic variants in THSD4, encoding the ADAMTS-like 6 protein, predispose to inherited thoracic aortic aneurysm

Purpose Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening disease with often unrecognized inherited forms. We sought to identify novel pathogenic variants associated with autosomal dominant inheritance of TAAD. Methods We analyzed exome sequencing data from 35 French TAAD families and performed next-generation sequencing capture panel of genes in 1114 unrelated TAAD patients. Functional effects of pathogenic variants identified were validated in cell, tissue, and mouse models. Results We identified five functional variants inTHSD4of which two heterozygous variants lead to a premature termination codon.THSD4encodes ADAMTSL6 (member of the ADAMTS/L superfamily), a microfibril-associated protein that promotes fibrillin-1 matrix assembly. TheTHSD4variants studied lead to haploinsufficiency or impaired assembly of fibrillin-1 microfibrils.Thsd4(+/-)mice showed progressive dilation of the thoracic aorta. Histologic examination of aortic samples from a patient carrying aTHSD4variant and fromThsd4(+/-)mice, revealed typical medial degeneration and diffuse disruption of extracellular matrix. Conclusion These findings highlight the role of ADAMTSL6 in aortic physiology and TAAD pathogenesis. They will improve TAAD management and help develop new targeted therapies

Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases

Mutations of the human valosin-containing protein gene cause autosomal-dominant inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia. We identified strumpellin as a novel valosin-containing protein binding partner. Strumpellin mutations have been shown to cause hereditary spastic paraplegia. We demonstrate that strumpellin is a ubiquitously expressed protein present in cytosolic and endoplasmic reticulum cell fractions. Overexpression or ablation of wild-type strumpellin caused significantly reduced wound closure velocities in wound healing assays, whereas overexpression of the disease-causing strumpellin N471D mutant showed no functional effect. Strumpellin knockdown experiments in human neuroblastoma cells resulted in a dramatic reduction of axonal outgrowth. Knockdown studies in zebrafish revealed severe cardiac contractile dysfunction, tail curvature and impaired motility. The latter phenotype is due to a loss of central and peripheral motoneuron formation. These data imply a strumpellin loss-of-function pathogenesis in hereditary spastic paraplegia. In the human central nervous system strumpellin shows a presynaptic localization. We further identified strumpellin in pathological protein aggregates in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia, various myofibrillar myopathies and in cortical neurons of a Huntington's disease mouse model. Beyond hereditary spastic paraplegia, our findings imply that mutant forms of strumpellin and valosin-containing protein may have a concerted pathogenic role in various protein aggregate diseases