Publications by authors named "J J Pedersen"

Introduction/aims: Primary hypokalemic periodic paralysis (HypoPP) can present with periodic paralysis and/or permanent muscle weakness. Permanent weakness is accompanied by fat replacement of the muscle. It is unknown whether the permanent muscle weakness is solely due to fat replacement or if other factors affect the ability of the remaining muscle fibers to contract.

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Background: Venous cannulation is widely used in healthcare systems, and for many patients, it is painful and distressing. We hypothesized that the rapid onset of cryospray use would reduce pain from venous cannulation compared to the use of a placebo spray.

Methods: The trial was a prospective randomized placebo-controlled trial including 130 adult patients scheduled for elective surgery.

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The central nervous system represents a major target tissue for therapeutic approach of numerous lysosomal storage disorders. Fabry disease arises from the lack or dysfunction of the lysosomal alpha-galactosidase A (GLA) enzyme, resulting in substrate accumulation and multisystemic clinical manifestations. Current enzyme replacement therapies (ERTs) face limited effectiveness due to poor enzyme biodistribution in target tissues and inability to reach the brain.

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Prenylation consists of the modification of proteins with either farnesyl diphosphate (FPP) or geranylgeranyl diphosphate (GGPP) at a cysteine near the C-terminus of target proteins to generate thioether-linked lipidated proteins. In recent work, metabolic labeling with alkyne-containing isoprenoid analogues including C15AlkOPP has been used to identify prenylated proteins and track their levels in different diseases. Here, a systematic study of the impact of isoprenoid length on proteins labeled with these probes was performed.

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Article Synopsis
  • Primary hypokalemic periodic paralysis (HypoPP) is a muscle condition that causes episodes of paralysis and can lead to permanent muscle weakness; understanding its natural progression could help in treatment evaluation.* -
  • A study examined 37 individuals with HypoPP using MRI to measure fat replacement in muscles and strength tests to track changes over 20 months, revealing increased fat in many muscles but no significant muscle strength changes.* -
  • The findings suggest that MRI is an effective tool for detecting subclinical progression of HypoPP, helping to assess disease progression in both symptomatic and asymptomatic patients.*
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