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Let’s talk about communication in strategic communication; : an analysis of students’ understanding of strategic communication and its implications

The aim of this paper is to address the importance of communication theory for the understanding of strategic communication. Underlying this focus are the debates and discussions on the definition of strategic communication, which have primarily been occupied with the meaning of the concept strategic. The definition and meaning of communication tend to be implicit or undefined and Van Ruler (2018)

In governments we trust : Enacting trust in Finnish and Swedish state-owned enterprises

The Swedish and Finnish states are significant owners of enterprises. In both countries, the results for the state-owned enterprises (SOE) are compiled into an annual report presented to the Parliament. It is a way to report on financial performance as well as a way to build trust and legitimize state ownership. The document is thus a meeting point and place of negotiation foreconomic and politica

Pronominal case in Västerbottnian

In this article, I consider the use of seemingly old nominative forms (ONF) in object position. The phenomenon is noted for the dialects of Västerbotten (in Northern Sweden) by several authors. Earlier works imply that the phenomenon is rather recent (early 1900s) and that the phenomenon is in more common usage in the inland rural areas than by the more urban coast. I discuss the structural analysIn this article, I consider the use of seemingly old nominative forms (ONF) in object position. The phenomenon is noted for the dialects of Västerbotten (in Northern Sweden) by several authors. Earlier works imply that the phenomenon is rather recent (early 1900s) and that the phenomenon is in more common usage in the inland rural areas than by the more urban coast. I discuss the structural analys

De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus

Subcellular membrane systems are highly enriched in dolichol, whose role in organelle homeostasis and endosomal-lysosomal pathway remains largely unclear besides being involved in protein glycosylation. DHDDS encodes for the catalytic subunit (DHDDS) of the enzyme cis-prenyltransferase (cis-PTase), involved in dolichol biosynthesis and dolichol-dependent protein glycosylation in the endoplasmic re

COG8 deficiency causes new congenital disorder of glycosylation type IIh

We describe a new Type II congenital disorder of glycosylation (CDG-II) caused by mutations in the conserved oligomeric Golgi (COG) complex gene, COG8. The patient has severe psychomotor retardation, seizures, failure to thrive and intolerance to wheat and dairy products. Analysis of serum transferrin and total serum N-glycans showed normal addition of one sialic acid, but severe deficiency in sub

Intracranial hemorrhage as the initial manifestation of a congenital disorder of glycosylation

Intracranial hemorrhage in a term neonate is a rare event in the absence of an identifiable precipitating factor such as severe thrombocytopenia, mechanical trauma, asphyxia, infections, or congenital vascular malformations. Congenital disorders of glycosylation are a genetically and clinically heterogeneous group of multisystem disorders characterized by the abnormal glycosylation of a number of

Reduced production of sulfated glycosaminoglycans occurs in Zambian children with kwashiorkor but not marasmus

BACKGROUND: Kwashiorkor, a form of severe malnutrition with high mortality, is characterized by edema and systemic abnormalities. Although extremely common, its pathophysiology remains poorly understood, and its characteristic physical signs are unexplained.OBJECTIVE: Because kwashiorkor can develop in protein-losing enteropathy, which is caused by a loss of enterocyte heparan sulfate proteoglycan

CDG-Id in two siblings with partially different phenotypes

We present two sibs with congenital disorder of glycosylation (CDG) type Id. Each shows severe global delay, failure to thrive, seizures, microcephaly, axial hypotonia, and disaccharidase deficiency. One sib has more severe digestive issues, while the other is more neurologically impaired. Each is compound heterozygous for a novel point mutation and an already known mutation in the ALG3 gene that

Ablation of mouse phosphomannose isomerase (Mpi) causes mannose 6-phosphate accumulation, toxicity, and embryonic lethality

MPI encodes phosphomannose isomerase, which interconverts fructose 6-phosphate and mannose 6-phosphate (Man-6-P), used for glycoconjugate biosynthesis. MPI mutations in humans impair protein glycosylation causing congenital disorder of glycosylation Ib (CDG-Ib), but oral mannose supplements normalize glycosylation. To establish a mannose-responsive mouse model for CDG-Ib, we ablated Mpi and provid

Congenital disorder of glycosylation Ic due to a de novo deletion and an hALG-6 mutation

We describe a new cause of congenital disorder of glycosylation-Ic (CDG-Ic) in a young girl with a rather mild CDG phenotype. Her cells accumulated lipid-linked oligosaccharides lacking three glucose residues, and sequencing of the ALG6 gene showed what initially appeared to be a homozygous novel point mutation (338G>A). However, haplotype analysis showed that the patient does not carry any patern

Hydrophobic Man-1-P derivatives correct abnormal glycosylation in Type I congenital disorder of glycosylation fibroblasts

Patients with Type I congenital disorders of glycosylation (CDG-I) make incomplete lipid-linked oligosaccharides (LLO). These glycans are poorly transferred to proteins resulting in unoccupied glycosylation sequons. Mutations in phosphomannomutase (PMM2) cause CDG-Ia by reducing the activity of PMM, which converts mannose (Man)-6-P to Man-1-P before formation of GDP-Man. These patients have reduce

Clinical and molecular characterization of the first adult congenital disorder of glycosylation (CDG) type Ic patient

Congenital disorder of glycosylation (CDG) type Ic, the second largest subtype of CDG, is caused by mutations in human ALG6 (hALG6). This gene encodes the alpha1,3-glucosyltransferase that catalyzes transfer of the first glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. In this report, we describe the first adult patient diagnosed with CDG-Ic, carrying two p