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The loss of longitudinal connectivity in regulated rivers, both up- and downstream, has been detrimental for biodiversity worldwide. While progress has been made regarding upstream fish passage solutions, many questions remain unanswered regarding downstream passage alternatives. To address these knowledge gaps, we used Atlantic salmon (S. salar) smolts to experimentally assess the guidance effici

Urban green and blue infrastructure (GBI) and associated ecosystem services (ES) are increasingly recognized for their potential to address urban sustainability challenges. This has resulted in an increase in studies quantifying ES in cities. In this review, we analysed 850 peer-reviewed publications to characterize urban ES assessment in terms of geographical location, urban GBI type and methodol

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)

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

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

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

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 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

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

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

We describe a case of congenital disorder of glycosylation with chronic diarrhea, progressive liver cirrhosis, and recurrent infections. Transferrin analysis showed only hyposialylation, but analysis of total serum N-glycans indicated loss of additional sugars, arguing that the latter generates a more informative picture to search for the primary defect.

We present the clinical, molecular, and biochemical diagnosis of a patient with congenital disorder of glycosylation (CDG)-Ih. We report significant brain dysfunction in this multisystem disease, further expanding its complex clinical spectrum.

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

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