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Congenital disorders of glycosylation. Part I. Defects of protein N-glycosylation

100%

Cylwik B. , Naklicki M. , Chrostek L. , Gruszewska E.

Acta Biochimica Polonica

2013

tom 60

nr 2

Glycosylation is the most common chemical process of protein modification and occurs in every living cell. Disturbances of this process may be either congenital or acquired. Congenital disorders of glycosylation (CDG) are a rapidly growing disease family, with about 50 disorders reported since its first clinical description in 1980. Most of the human diseases have been discovered recently. CDG result from defects in the synthesis of the N- and O-glycans moiety of glycoproteins, and in the attachment to the polypeptide chain of proteins. These defects have been found in the activation, presentation, and transport of sugar precursors, in the enzymes responsible for glycosylation, and in proteins that control the traffic of component. There are two main types of protein glycosylation: N-glycosylation and O-glycosylation. Most diseases are due to defects in the N-glycosylation pathway. For the sake of convenience, CDG were divided into 2 types, type I and II. CDG can affect nearly all organs and systems. The considerable variability of clinical features makes it difficult to recognize patients with CDG. Diagnosis can be made on the basis of abnormal glycosylation display. In this paper, an overview of CDG with a new nomenclature limited to the group of protein N-glycosylation disorders, clinical phenotype and diagnostic approach, have been presented. The location, reasons for defects, and the number of cases have been also described. This publication aims to draw attention to the possibility of occurrence of CDG in each multisystem disorder with an unknown origin.

The impact of graphite impact on the thermal conductivity of the solidified grout

63%

Śliwa T. , Kowalski T. , Stryczek S. , Wiśniowski D. , Bieda A. , Piwowarczyk S. , Beszłej J. , Naklicki M. , Sapińska-Śliwa A.

AGH Drilling, Oil, Gas

2017

tom Vol. 34, no. 4

811--820

The fast development of low-temperature geothermal energy affects the search for newer and newer solutions and materials used in the construction of borehole heat exchangers. One of the most important factors affecting the correct operation of heat pump systems with borehole heat exchangers is appropriately selected solidified grout. Solidifies grout has to be characterized by the highest thermal conductivity and good sealing borehole heat exchangers. The paper will present the impact of graphite on the thermal conductivity of solidified grout.

The impact of diatomite on the thermal conductivity of solidified grout

63%

Dubiel S. , Śliwa T. , Kowalski T. , Stryczek S. , Wiśniowski D. , Bieda A. , Piwowarczyk S. , Beszłej J. , Naklicki M.

AGH Drilling, Oil, Gas

2017

tom Vol. 34, no. 3

691--700

The use of suitable materials for the preparation of sealing grout is an important issue from the point of view of the correct construction of borehole heat exchangers and geothermal wells. The minimization of the loss of heat during the transport of thermal water in geothermal wells can be achieved by use of sealing slurries with reduced thermal conductivity. Such slurries are also recommended in the upper part of deep borehole heat exchangers and the upper part of energy piles. The publication describes the impact of the addition of diatomite as a material lowering the thermal conductivity of solidified grout.