Is the employed scholar free not to publish?:Limitations of disclosure rights in a comparative perspective

The control of works produced by academics in the course of their employment is a controversial issue. This paper examines the protection offered to employed scholars who do not want to publish their work because of the fear that premature dissemination would damage their academic reputation. The right not to publish of employed scholars has been analyzed considering Anglo-American copyright law on the one hand, and French legislation on the other. Irrespective of the differences between these jurisdictions, both positions allow labour conditions to restrict the right not to publish. On top of the comparison of three legal systems, this paper investigates the question of whether the limitations on the right of disclosure conflict with article 15, paragraph 1 c of the International Covenant on Economic, Social and Cultural Rights ICESCR. Both Anglo-American and French copyright law are not fully consistent with the protection of moral interests offered by the ICESCR. The reason is that it depends on the labour conditions whether there exists any obligation on academic employees to publish. In the absence of this obligation, the employed scholar enjoys the freedom to decide not to publish. ICESCR does not allow these limitations of disclosure rights since article 15, paragraph 1 c does not refer to working conditions

Expanding the clinical spectrum of biglycan-related Meester-Loeys syndrome

\ua9 The Author(s) 2024.Pathogenic loss-of-function variants in BGN, an X-linked gene encoding biglycan, are associated with Meester-Loeys syndrome (MRLS), a thoracic aortic aneurysm/dissection syndrome. Since the initial publication of five probands in 2017, we have considerably expanded our MRLS cohort to a total of 18 probands (16 males and 2 females). Segregation analyses identified 36 additional BGN variant-harboring family members (9 males and 27 females). The identified BGN variants were shown to lead to loss-of-function by cDNA and Western Blot analyses of skin fibroblasts or were strongly predicted to lead to loss-of-function based on the nature of the variant. No (likely) pathogenic missense variants without additional (predicted) splice effects were identified. Interestingly, a male proband with a deletion spanning the coding sequence of BGN and the 5’ untranslated region of the downstream gene (ATP2B3) presented with a more severe skeletal phenotype. This may possibly be explained by expressional activation of the downstream ATPase ATP2B3 (normally repressed in skin fibroblasts) driven by the remnant BGN promotor. This study highlights that aneurysms and dissections in MRLS extend beyond the thoracic aorta, affecting the entire arterial tree, and cardiovascular symptoms may coincide with non-specific connective tissue features. Furthermore, the clinical presentation is more severe and penetrant in males compared to females. Extensive analysis at RNA, cDNA, and/or protein level is recommended to prove a loss-of-function effect before determining the pathogenicity of identified BGN missense and non-canonical splice variants. In conclusion, distinct mechanisms may underlie the wide phenotypic spectrum of MRLS patients carrying loss-of-function variants in BGN

Is the employed scholar free not to publish?: Limitations of disclosure rights in a comparative perspective

The control of works produced by academics in the course of their employment is a controversial issue. This paper examines the protection offered to employed scholars who do not want to publish their work because of the fear that premature dissemination would damage their academic reputation. The right not to publish of employed scholars has been analyzed considering Anglo-American copyright law on the one hand, and French legislation on the other. Irrespective of the differences between these jurisdictions, both positions allow labour conditions to restrict the right not to publish. On top of the comparison of three legal systems, this paper investigates the question of whether the limitations on the right of disclosure conflict with article 15, paragraph 1 c of the International Covenant on Economic, Social and Cultural Rights ICESCR. Both Anglo-American and French copyright law are not fully consistent with the protection of moral interests offered by the ICESCR. The reason is that it depends on the labour conditions whether there exists any obligation on academic employees to publish. In the absence of this obligation, the employed scholar enjoys the freedom to decide not to publish. ICESCR does not allow these limitations of disclosure rights since article 15, paragraph 1 c does not refer to working conditions

Expanding the clinical spectrum of biglycan-related Meester-Loeys syndrome

Pathogenic loss-of-function variants in BGN, an X-linked gene encoding biglycan, are associated with Meester-Loeys syndrome (MRLS), a thoracic aortic aneurysm/dissection syndrome. Since the initial publication of five probands in 2017, we have considerably expanded our MRLS cohort to a total of 18 probands (16 males and 2 females). Segregation analyses identified 36 additional BGN variant-harboring family members (9 males and 27 females). The identified BGN variants were shown to lead to loss-of-function by cDNA and Western Blot analyses of skin fibroblasts or were strongly predicted to lead to loss-of-function based on the nature of the variant. No (likely) pathogenic missense variants without additional (predicted) splice effects were identified. Interestingly, a male proband with a deletion spanning the coding sequence of BGN and the 5' untranslated region of the downstream gene (ATP2B3) presented with a more severe skeletal phenotype. This may possibly be explained by expressional activation of the downstream ATPase ATP2B3 (normally repressed in skin fibroblasts) driven by the remnant BGN promotor. This study highlights that aneurysms and dissections in MRLS extend beyond the thoracic aorta, affecting the entire arterial tree, and cardiovascular symptoms may coincide with non-specific connective tissue features. Furthermore, the clinical presentation is more severe and penetrant in males compared to females. Extensive analysis at RNA, cDNA, and/or protein level is recommended to prove a loss-of-function effect before determining the pathogenicity of identified BGN missense and non-canonical splice variants. In conclusion, distinct mechanisms may underlie the wide phenotypic spectrum of MRLS patients carrying loss-of-function variants in BGN.</p