Industrial Change in Old Port Areas, the Case of the Port of Toronto

The classical association of ports with industry needs revising. Traditionally, industry in the port area was concerned with the port operation itself, with ships, and with the cargoes transported by the ships. In recent years, as a result of technological changes, land use in the central water-front has tended to switch into residential, recreational, commercial and institutional uses, while new industries have been attracted to the adjacent old port-areas. These new industries have an entirely different role, being much more closely tied to the function of the city itself. They include: industry oriented to serving the local urban market; noxious industry; and labour-oriented industry. Many port-related industries are now located downriver or at deep-water greenfield locations where extensive sites are available. The illustration of these changes is presented with a case study of Toronto and the example of some major Canadian port cities.L'association classique entre les ports et les industries doit être réexaminée. Traditionnellement, les industries situées dans les zones portuaires étaient dépendantes du fonctionnement du port lui-même, des navires, et des marchandises transportées par ces navires. Récemment, par suite de changements technologiques, la zone située à proximité des quais centraux a vu apparaître des usages résidentiel, récréationnel, commercial et institutionnel, alors que de nouvelles industries ont été attirées par les vieilles zones portuaires qui sont contiguës. Ces nouvelles industries ont un rôle tout différent, car elles sont associées aux fonctions de la ville elle-même. Elles comprennent les industries associées au marché urbain régional, les industries nuisibles pour l'environnement et les industries étroitement dépendantes de la main-d'oeuvre. Beaucoup d'industries dépendant directement du port ont émigré dans les zones situées en aval ou dans des sites d'eau profonde à proximité d'espaces vacants. Ces changements sont illustrés à l'aide du cas du port de Toronto et de l'exemple de quelques grandes villes portuaires canadiennes

An overview of knowledge sharing in new product development

This paper provides an overview of some of the issues in knowledge management related to the sharing of knowledge in new product development. Previous research and concepts reported by international researchers, and examples of the research projects carried out by the authors will be introduced. The paper first provides an overview of the history and importance of innovation and challenges in manufacturing. Then the importance of new product development in the sustainable success of manufacturing enterprises in the globalised business operations is discussed. The formalisation and modelling of product development processes will also be introduced. The concept and different definitions of knowledge management by previous researchers are then introduced, with further discussion on knowledge sharing. At this point, the authors’ research in knowledge sharing is also introduced. Finally, the trend of using social media and Enterprise 2 technologies in knowledge management and sharing is introduced using the recent research projects of the authors as examples

Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture

The genetic basis of Lewy body dementia (LBD) is not well understood. Here, we performed whole-genome sequencing in large cohorts of LBD cases and neurologically healthy controls to study the genetic architecture of this understudied form of dementia, and to generate a resource for the scientific community. Genome-wide association analysis identified five independent risk loci, whereas genome-wide gene-aggregation tests implicated mutations in the gene GBA. Genetic risk scores demonstrate that LBD shares risk profiles and pathways with Alzheimer's disease and Parkinson's disease, providing a deeper molecular understanding of the complex genetic architecture of this age-related neurodegenerative condition

Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis.

We examined the role of repeat expansions in the pathogenesis of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) by analyzing whole-genome sequence data from 2,442 FTD/ALS patients, 2,599 Lewy body dementia (LBD) patients, and 3,158 neurologically healthy subjects. Pathogenic expansions (range, 40-64 CAG repeats) in the huntingtin (HTT) gene were found in three (0.12%) patients diagnosed with pure FTD/ALS syndromes but were not present in the LBD or healthy cohorts. We replicated our findings in an independent collection of 3,674 FTD/ALS patients. Postmortem evaluations of two patients revealed the classical TDP-43 pathology of FTD/ALS, as well as huntingtin-positive, ubiquitin-positive aggregates in the frontal cortex. The neostriatal atrophy that pathologically defines Huntington's disease was absent in both cases. Our findings reveal an etiological relationship between HTT repeat expansions and FTD/ALS syndromes and indicate that genetic screening of FTD/ALS patients for HTT repeat expansions should be considered

Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture

The genetic basis of Lewy body dementia (LBD) is not well understood. Here, we performed whole-genome sequencing in large cohorts of LBD cases and neurologically healthy controls to study the genetic architecture of this understudied form of dementia and to generate a resource for the scientific community. Genome-wide association analysis identified five independent risk loci, whereas genome-wide gene-aggregation tests implicated mutations in the gene GBA. Genetic risk scores demonstrate that LBD shares risk profiles and pathways with Alzheimer’s and Parkinson’s disease, providing a deeper molecular understanding of the complex genetic architecture of this age-related neurodegenerative condition

Genome sequencing analysis identifies new loci associated with Lewy body dementia and provides insights into its genetic architecture

The genetic basis of Lewy body dementia (LBD) is not well understood. Here, we performed whole-genome sequencing in large cohorts of LBD cases and neurologically healthy controls to study the genetic architecture of this understudied form of dementia, and to generate a resource for the scientific community. Genome-wide association analysis identified five independent risk loci, whereas genome-wide gene-aggregation tests implicated mutations in the gene GBA. Genetic risk scores demonstrate that LBD shares risk profiles and pathways with Alzheimer's disease and Parkinson's disease, providing a deeper molecular understanding of the complex genetic architecture of this age-related neurodegenerative condition

Cardiac-locked bursts of muscle sympathetic nerve activity are absent in familial dysautonomia

Familial dysautonomia (Riley–Day syndrome) is an hereditary sensory and autonomic neuropathy (HSAN type III), expressed at birth, that is associated with reduced pain and temperature sensibilities and absent baroreflexes, causing orthostatic hypotension as well as labile blood pressure that increases markedly during emotional excitement. Given the apparent absence of functional baroreceptor afferents, we tested the hypothesis that the normal cardiac-locked bursts of muscle sympathetic nerve activity (MSNA) are absent in patients with familial dysautonomia. Tungsten microelectrodes were inserted percutaneously into muscle or cutaneous fascicles of the common peroneal nerve in 12 patients with familial dysautonomia. Spontaneous bursts of MSNA were absent in all patients, but in five patients we found evidence of tonically firing sympathetic neurones, with no cardiac rhythmicity, that increased their spontaneous discharge during emotional arousal but not during a manoeuvre that unloads the baroreceptors. Conversely, skin sympathetic nerve activity (SSNA), recorded in four patients, appeared normal. We conclude that the loss of phasic bursts of MSNA and the loss of baroreflex modulation of muscle vasoconstrictor drive contributes to the poor control of blood pressure in familial dysautonomia, and that the increase in tonic firing of muscle vasoconstrictor neurones contributes to the increase in blood pressure during emotional excitement

Relationship between proprioception at the knee joint and gait ataxia in HSAN III

Background: Hereditary sensory and autonomic neuropathy type III features marked ataxic gait that progressively worsens over time. We assessed whether proprioceptive disturbances can explain the ataxia. Methods: Proprioception at the knee joint was assessed using passive joint angle matching in 18 patients and 14 age-matched controls; 5 patients with cerebellar ataxia were also studied. Ataxia was quantified using the Brief Ataxia Rating Score, which ranged from 7 to 26 of 30. Results: Neuropathy patients performed poorly in judging joint position: mean absolute error was 8.7°±1.0°, and the range was very wide (2.8°-18.1°); conversely, absolute error was only 2.7°±0.3° (1.6°-5.5°) in the controls and 3.0°±0.2° (2.1°-3.4°) in the cerebellar patients. This error was positively correlated to the degree of ataxia in the neuropathy patients but not the cerebellar patients. Conclusions: These results suggest that poor proprioceptive acuity at the knee joint is a major contributor to the ataxic gait associated with hereditary sensory and autonomic neuropathy type III