--- abstract: "Two consanguineous families with Uner Tan Syndrome (UTS) were analyzed in relation to self-organizing processes in complex systems, and the evolutionary emergence of human\r\nbipedalism. The cases had the key symptoms of previously reported cases of UTS, such as quadrupedalism, mental retardation, and dysarthric or no speech, but the new cases\r\nalso exhibited infantile hypotonia and are designated UTS Type-II. There were 10 siblings in Branch I and 12 siblings in Branch II. Of these, there were seven cases exhibiting habitual quadrupedal locomotion (QL): four deceased and three living. The infantile hypotonia in the\r\nsurviving cases gradually disappeared over a period of years, so that they could sit by about 10 years, crawl on hands and knees by about 12 years. They began walking on all fours at around 14 years, habitually using QL. Neurological examinations showed normal tonus in\r\ntheir arms and legs, no Babinski sign, brisk tendon reflexes especially in the legs, and mild tremor. The patients could not walk in a straight line, but (except in one case) could stand up and maintain upright posture with truncal ataxia. Cerebello-vermial hypoplasia and mild gyral simplification were noted in their MRIs. The results of the genetic analysis were inconclusive: no genetic code could be identified as the triggering factor for the syndrome\r\nin these families. Instead, the extremely low socio-economic status of the patients was thought to play a role in the emergence of UTS, possibly by epigenetically changing the brain structure and function, with a consequent selection of ancestral neural networks for\r\nQL during locomotor development. It was suggested that UTS may be regarded as one of the unpredictable outcomes of self-organization within a complex system. It was also\r\nnoted that the prominent feature of this syndrome, the diagonal-sequence habitual QL, generated an interference between ipsilateral hands and feet, as innon-humanprimates. It was suggested that this may have been the triggering factor for the attractor state “bipedal locomotion” (BL), which had visual and manual benefits for our ape-like ancestors, and therefore enhancing their chances for survival, with consequent developments in the psychomotor domain of humans. This was put forward as a novel theory of the evolution of BL in human beings." altloc: [] chapter: ~ commentary: ~ commref: ~ confdates: ~ conference: ~ confloc: ~ contact_email: ~ creators_id: [] creators_name: - family: Tan given: Uner honourific: Prof. Dr. lineage: '' date: 2014 date_type: published datestamp: 2014-05-10 00:39:06 department: ~ dir: disk0/00/00/96/86 edit_lock_since: ~ edit_lock_until: 0 edit_lock_user: ~ editors_id: [] editors_name: - family: Williams given: J. Michael honourific: Dr. lineage: '' eprint_status: archive eprintid: 9686 fileinfo: /9686/1.hassmallThumbnailVersion/UTS%20ARTICLE%20and%20NEW%20THEORY%20on%20EVOLUTION%20OF%20BIPEDALISM%202014.pdf;/9686/1/UTS%20ARTICLE%20and%20NEW%20THEORY%20on%20EVOLUTION%20OF%20BIPEDALISM%202014.pdf full_text_status: public importid: ~ institution: ~ isbn: ~ ispublished: pub issn: ~ item_issues_comment: [] item_issues_count: ~ item_issues_description: [] item_issues_id: [] item_issues_reported_by: [] item_issues_resolved_by: [] item_issues_status: [] item_issues_timestamp: [] item_issues_type: [] keywords: 'Uner Tan syndrome, ataxia, quadrupedalism, evolution, complex systems, self-organization' lastmod: 2015-04-20 11:45:42 latitude: ~ longitude: ~ metadata_visibility: show note: ~ number: 84 pagerange: ~ pubdom: TRUE publication: Frontiers in Neuroscience publisher: www.frontiers.org refereed: TRUE referencetext: "Bem, T., Cabelguen, J-M., Ekeberg, Ö., Grillner, S. 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D., Hurst, J. W. (Eds.). \r\n Clinical methods: the history, physical, and \r\n laboratory examinations, 3rd edition. Boston: \r\n Butterworths. \r\nYoung, J. W. (2012). Gait selection and the ontogeny of \r\n quadrupedal walking in squirrel monkeys (Saimiri \r\n boliviensis). Am. J. Phys. Anthropol. 147, 580- \r\n 582. \r\nZehr, E. P., Hundza, S. R., and Vasudevan, E. V. (2009). \r\n The quadrupedal nature of human bipedal \r\n locomotion. Exerc. Sport Sci. Rev. 37, 102-108. \r\n" relation_type: [] relation_uri: [] reportno: ~ rev_number: 11 series: ~ source: ~ status_changed: 2014-05-10 00:39:06 subjects: - neuro-neu succeeds: ~ suggestions: "Two closely related consanguineous families with siblings exhibiting UTS were presented. They lived in a small village near Diyarbakir, in South-Eastern Turkey. The affected siblings had infantile hypotonia, which had gradually disappeared by adolescence, and was replaced by QL during adulthood (UTS Type-II: a novel variant of UTS). No mutation was found in the VLDLR gene, but the genetic analysis was inconclusive. No single gene had so far been identified as a single factor directly involved in the emergence of UTS; consanguinity was not a prerequisite for the emergence of the syndrome, consistent with the Editorial in Nature Genetics: “it is unlikely that consanguinity contributes significantly to polygenic and multifactorial disease once socio-economic variables have been controlled for.” The only factor shared by all of the UTS families was their low socio-economic status, which may have led to epigenetic changes during embryogenesis, with postnatal psychomotor impairments extending from childhood to adulthood. The patients exhibited diagonal-sequence QL similar to non-human primates. The first appearing fish-like tetrapods during the Devonian period also used diagonal-sequence QL, nearly 400 MYA, suggesting that the neural networks, such as the spinal central pattern generators, for this kind of QL were preserved throughout the evolution of tetrapods, and human beings are still using the neural networks for diagonal-sequence QL. Non-primates with lateral-sequence QL did not show such an evolutionary course. \r\n The diagonal-sequence QL was accompanied by interference between the ipsilateral hands and feet in patients, similar to that also found in non-human primates, which would be disadvantageous for effective locomotion. It was suggested that this locomotor disadvantage may have had evolutionarily advantageous consequences for human beings. Namely, the evolutionary development of human bipedalism may have been the result of long-lasting adaptive self-organizing processes to overcome the interference effect of quadrupedalism. The attractor state, bipedalism, could then be achieved, which would free the hands from locomotion, making them more suitable for skilled actions. This is the ipsilateral limb interference theory for the evolution of human bipedalism, put forward for the first time in the present work. This may be plausible if the evolution is considered as the outcome of self-organization with unpredictable attractor states, occurring not under the influence of previously established programs or single factors, such as neural and/or genetic codes (see also Tan, 2010). The beneficial effects of habitual BL would then enhance the chances for survival and further developments in the psychomotor domain of human beings. \r\n" sword_depositor: ~ sword_slug: ~ thesistype: ~ title: "Two families with quadrupedalism, mental retardation, no\r\nspeech, and infantile hypotonia (Uner Tan Syndrome\r\nType-II); a novel theory for the evolutionary emergence of\r\nhuman bipedalism" type: journale userid: 4979 volume: 8