As Below So Above: Reconstructing the Neo-Babylonian Worldview

To add to our knowledge about a Near Eastern culture, this project examines through textual evidence how the early first millennium BCE Neo-Babylonians thought, reasoned, and wrote in order to partially reconstruct the shared, generally held worldview of the Neo-Babylonian people using the transdisciplinary approach of worldview analysis. Worldviews are what we use to think with, not what we think about. Underlying surficial cultural behaviors are deeper levels of cognition regarding how to reason, perceive the world, prioritize values, prescribe behavior, and explain all of life. Specifically, this work examines the language and logic reflected in the textual archive, believing that this is the foundational level of any worldview. I argue that one finds two related components: (1) that they were linguistically programmed to be attuned to the full context over particularities, verbal actions over agential subjects, the continuity of substances over discrete objects, the standard use of maleness over femaleness, and the affective power of spoken or written words, and (2) that they were logically programmed to prefer gradations over distinctions, functional properties over inherent attributes, radials and/or rhizomes over linearity, and relationships and/or comparisons over abstractions and algorithms. By addressing this underlying, implicit cognitive software the Neo-Babylonians used, one is better able to understand the society’s more observable and obvious religious, ethical, legal, political, and social features. This has the potential to present a more contextualized view of Neo-Babylonian civilization. Reconstructing the ancient Neo-Babylonian worldview allows scholars to compare and contrast the linguistic and logical features to other ancient nearby cultures in order to understand continuities and differences and what accounts for them. One can open a dialogue between these ancient societies at a deeper level. It demonstrates the uniqueness of Neo-Babylonia. And it provides a basis for understanding how Neo-Babylonians contributed to the roots of Western civilization and thought. Most current worldview analysis examines modern or postmodern worldviews. By examining an ancient worldview, one can begin to more clearly understand any common aspects which exist for all worldviews and any elements that exist in ancient ones which are missing from cataloging more modern worldviews. Thus, the cataloging of an ancient worldview helps to open new vistas within worldview studies. This study invites similar ones within ancient Near Eastern studies and within ancient studies in general

KCTD7 deficiency defines a distinct neurodegenerative disorder with a conserved autophagy-lysosome defect

International audienceOBJECTIVE:Several small case series identified KCTD7 mutations in patients with a rare autosomal recessive disorder designated progressive myoclonic epilepsy (EPM3) and neuronal ceroid lipofuscinosis (CLN14). Despite the name KCTD (potassium channel tetramerization domain), KCTD protein family members lack predicted channel domains. We sought to translate insight gained from yeast studies to uncover disease mechanisms associated with deficiencies in KCTD7 of unknown function.METHODS:Novel KCTD7 variants in new and published patients were assessed for disease causality using genetic analyses, cell-based functional assays of patient fibroblasts and knockout yeast, and electron microscopy of patient samples.RESULTS:Patients with KCTD7 mutations can exhibit movement disorders or developmental regression before seizure onset, and are distinguished from similar disorders by an earlier age of onset. Although most published KCTD7 patient variants were excluded from a genome sequence database of normal human variations, most newly identified patient variants are present in this database, potentially challenging disease causality. However, genetic analysis and impaired biochemical interactions with cullin 3 support a causal role for patient KCTD7 variants, suggesting deleterious alleles of KCTD7 and other rare disease variants may be underestimated. Both patient-derived fibroblasts and yeast lacking Whi2 with sequence similarity to KCTD7 have impaired autophagy consistent with brain pathology.INTERPRETATION:Biallelic KCTD7 mutations define a neurodegenerative disorder with lipofuscin and lipid droplet accumulation but without defining features of neuronal ceroid lipofuscinosis or lysosomal storage disorders. KCTD7 deficiency appears to cause an underlying autophagy-lysosome defect conserved in yeast, thereby assigning a biological role for KCTD7