The role of human factors in the mechanism of innovative software companies

У статті досліджено значення та взаємозв’язок людини, як складової механізму інноваційного забезпечення підприємств, з іншими ресурсами підприємства на всіх рівнях.«Přední vědecké novinky»: IX sběrné nádobě obsahují materiály mezinárodní vědecko–praktická konference «Přední vědecké novinky» (27 srpna - 05 září 2013 roku) posekcích «Ekonomické vědy» – С.87–96

Pattern of expression and relative proportions of the human splice variants of region 8–10.

<p>(A) Upper panel: schematic representation of the different splicing events that can occur in this region. Lower panel: relative quantities of these variants in a panel of human tissues, expressed as a percentage of total WNK1 expression. Cereb.: Cerebellum; Sp. cord: spinal cord; DRG: Dorsal Root Ganglia; Sk. m.: Skeletal muscle. (B) A HSN2-containing WNK1 transcript is expressed in mDCT cells. RT-QPCR was performed between exon 8 and HSN2, resulting in the amplification of a 228 bp fragment (arrow), corresponding to the “Δ8b” isoform, and a 486 bp fragment (arrowhead), corresponding to the complete isoform. RNA extracts from mouse brain, expressing both isoforms, and from mouse kidney, expressing mostly the “Δ8b” isoform, were used as control. The weak band indicated by an asterisk results from a non-specific amplification (verified by sequencing). (C) The HSN2-containing protein is detected in the mouse kidney. Immunoblot of various mouse tissues incubated with an antibody directed against the portion of the protein encoded by HSN2. A strong signal is detected in the spinal cord and a lower signal in the brain and renal cortex. An immunoblot incubated with a HSP90 antibody shows equal loading of the different samples.</p

Computed tomography angiography in a patient with medial dibromuscular dysplasia

<p><b>Copyright information:</b></p><p>Taken from "Fibromuscular dysplasia"</p><p>http://www.OJRD.com/content/2/1/28</p><p>Orphanet Journal of Rare Diseases 2007;2():28-28.</p><p>Published online 7 Jun 2007</p><p>PMCID:PMC1899482.</p><p></p

Missense mutation distribution in collagen III.

<p>Missense mutation frequency along the residues of the collagen III chain. This demonstrates the non-random distribution of variation.</p

The collαgen III fibril has a "flexi-rod" structure of flexible sequences interspersed with rigid bioactive domains including two with hemostatic roles.

Collagen III is critical to the integrity of blood vessels and distensible organs, and in hemostasis. Examination of the human collagen III interactome reveals a nearly identical structural arrangement and charge distribution pattern as for collagen I, with cell interaction domains, fibrillogenesis and enzyme cleavage domains, several major ligand-binding regions, and intermolecular crosslink sites at the same sites. These similarities allow heterotypic fibril formation with, and substitution by, collagen I in embryonic development and wound healing. The collagen III fibril assumes a "flexi-rod" structure with flexible zones interspersed with rod-like domains, which is consistent with the molecule's prominence in young, pliable tissues and distensible organs. Collagen III has two major hemostasis domains, with binding motifs for von Willebrand factor, α2β1 integrin, platelet binding octapeptide and glycoprotein VI, consistent with the bleeding tendency observed with COL3A1 disease-causing sequence variants

Arrangement of five overlapping collagen III α1 chains in the fibril D-period with overlap and gap zone indicated.

<p>Arrangement of five overlapping collagen III α1 chains in the fibril D-period with overlap and gap zone indicated.</p

Arrangement of collagen I α1 (top), α2 (middle) and collagen III α1 (bottom) chains demonstrates approximate alignment of charge across all three molecules in the D-period.

<p>Arrangement of collagen I α1 (top), α2 (middle) and collagen III α1 (bottom) chains demonstrates approximate alignment of charge across all three molecules in the D-period.</p

Major structural features and ligand binding sites for collagen III.

<p>Major structural features and ligand binding sites for collagen III.</p

Table_3_Assessment of arterial damage in vascular Ehlers-Danlos syndrome: A retrospective multicentric cohort.DOCX

BackgroundVascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder due to pathogenic variants in COL3A1 leading to medium-size-artery (MSA) dissection, aneurysm, rupture. Aortic lesions are rarer and less investigated. The objective was to describe the distribution of MSA and aortic lesions and the type of COL3A1 variants in a multicentric cohort of 330 adult vEDS patients.MethodsAt the time of the study, 87% were alive, 60.3% were index cases, and 60.0% were women. COL3A1 variants were identified using NGS and/or Sanger sequencing and classified according to functional consequences: 80.6% leading to dominant-negative (DN) and 19.4% leading to haploinsufficiency (HI). Imaging was systematically performed during the initial workup. Carotid mechanics were assessed by echo tracking in a subgroup of patients.ResultsArterial lesions were reported in 82.4% of the patients (N = 272): 83.5% had MSA lesions alone, 3.3% had aortic lesions alone, and 13.2% both. DN variants were associated with a higher prevalence of arterial lesions (P ConclusionThe prevalence of aortic lesions is not influenced by the COL3A1 genotype when adjusted for age. Patients with DN variant vEDS have a higher frequency of MSA lesions, especially in supra-aortic trunks associated with lower carotid stiffness. These results support optimized care and follow-up for these vulnerable patients.</p

Table_1_Assessment of arterial damage in vascular Ehlers-Danlos syndrome: A retrospective multicentric cohort.XLSX

BackgroundVascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder due to pathogenic variants in COL3A1 leading to medium-size-artery (MSA) dissection, aneurysm, rupture. Aortic lesions are rarer and less investigated. The objective was to describe the distribution of MSA and aortic lesions and the type of COL3A1 variants in a multicentric cohort of 330 adult vEDS patients.MethodsAt the time of the study, 87% were alive, 60.3% were index cases, and 60.0% were women. COL3A1 variants were identified using NGS and/or Sanger sequencing and classified according to functional consequences: 80.6% leading to dominant-negative (DN) and 19.4% leading to haploinsufficiency (HI). Imaging was systematically performed during the initial workup. Carotid mechanics were assessed by echo tracking in a subgroup of patients.ResultsArterial lesions were reported in 82.4% of the patients (N = 272): 83.5% had MSA lesions alone, 3.3% had aortic lesions alone, and 13.2% both. DN variants were associated with a higher prevalence of arterial lesions (P ConclusionThe prevalence of aortic lesions is not influenced by the COL3A1 genotype when adjusted for age. Patients with DN variant vEDS have a higher frequency of MSA lesions, especially in supra-aortic trunks associated with lower carotid stiffness. These results support optimized care and follow-up for these vulnerable patients.</p