FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2557382017> ?p ?o ?g. }

• W2557382017 abstract "Abstract Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs). Historically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegeneration with death in the first decade due to cardio-pulmonary complications. New data suggest that inflammation may play a role in MPS pathophysiology. To date there is almost no information on the pathophysiologic changes within the cerebral spinal fluid (CSF) of these patients. We evaluated the CSF of 25 consecutive patients with MPS-IH. While CSF glucose and total protein were within the normal range, we found a significantly mean elevated CSF opening pressure at 24 cm H 2 O (range 14–37 cm H 2 O). We observed a 3-fold elevation in CSF heparan sulfate and a 3–8 fold increase in MPS-IH specific non-reducing ends, I0S0 and I0S6. Cytokine analyses in CSF of children with MPS-IH showed significantly elevated inflammatory markers including: MCP-1 SDF-1a, IL-Ra, MIP-1b, IL-8, and VEGF in comparison to unaffected children. This is the largest report of CSF characteristics in children with MPS-IH. Identification of key biomarkers may provide further insight into the inflammatory-mediated mechanisms related to MPS diseases and perhaps lead to improved targeted therapies." @default.
• W2557382017 created "2016-12-08" @default.
• W2557382017 creator A5010997422 @default.
• W2557382017 creator A5011104827 @default.
• W2557382017 creator A5014639393 @default.
• W2557382017 creator A5033661466 @default.
• W2557382017 creator A5051468151 @default.
• W2557382017 creator A5077588523 @default.
• W2557382017 creator A5085708802 @default.
• W2557382017 date "2016-12-02" @default.
• W2557382017 modified "2023-10-11" @default.
• W2557382017 title "Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H" @default.
• W2557382017 cites W1563374141 @default.
• W2557382017 cites W1585492421 @default.
• W2557382017 cites W1605694349 @default.
• W2557382017 cites W1747533618 @default.
• W2557382017 cites W1966811644 @default.
• W2557382017 cites W1967296273 @default.
• W2557382017 cites W1968067472 @default.
• W2557382017 cites W1987561426 @default.
• W2557382017 cites W1988681265 @default.
• W2557382017 cites W1989186343 @default.
• W2557382017 cites W2001101692 @default.
• W2557382017 cites W2001848682 @default.
• W2557382017 cites W2004927707 @default.
• W2557382017 cites W2007804178 @default.
• W2557382017 cites W2015226637 @default.
• W2557382017 cites W2019263584 @default.
• W2557382017 cites W2021058027 @default.
• W2557382017 cites W2026066934 @default.
• W2557382017 cites W2028137014 @default.
• W2557382017 cites W2031042559 @default.
• W2557382017 cites W2033181927 @default.
• W2557382017 cites W2036725915 @default.
• W2557382017 cites W2043754477 @default.
• W2557382017 cites W2070980825 @default.
• W2557382017 cites W2089713475 @default.
• W2557382017 cites W2089990110 @default.
• W2557382017 cites W2099257972 @default.
• W2557382017 cites W2121971502 @default.
• W2557382017 cites W2126590211 @default.
• W2557382017 cites W2127971440 @default.
• W2557382017 cites W2147129577 @default.
• W2557382017 cites W2151879267 @default.
• W2557382017 cites W2158514667 @default.
• W2557382017 cites W2159823307 @default.
• W2557382017 cites W2263658783 @default.
• W2557382017 cites W2270089697 @default.
• W2557382017 cites W2303018029 @default.
• W2557382017 cites W2393713187 @default.
• W2557382017 cites W4236644412 @default.
• W2557382017 doi "https://doi.org/10.1038/srep38305" @default.
• W2557382017 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5133554" @default.
• W2557382017 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27910891" @default.
• W2557382017 hasPublicationYear "2016" @default.
• W2557382017 type Work @default.
• W2557382017 sameAs 2557382017 @default.
• W2557382017 citedByCount "23" @default.
• W2557382017 countsByYear W25573820172017 @default.
• W2557382017 countsByYear W25573820172018 @default.
• W2557382017 countsByYear W25573820172019 @default.
• W2557382017 countsByYear W25573820172020 @default.
• W2557382017 countsByYear W25573820172021 @default.
• W2557382017 countsByYear W25573820172022 @default.
• W2557382017 countsByYear W25573820172023 @default.
• W2557382017 crossrefType "journal-article" @default.
• W2557382017 hasAuthorship W2557382017A5010997422 @default.
• W2557382017 hasAuthorship W2557382017A5011104827 @default.
• W2557382017 hasAuthorship W2557382017A5014639393 @default.
• W2557382017 hasAuthorship W2557382017A5033661466 @default.
• W2557382017 hasAuthorship W2557382017A5051468151 @default.
• W2557382017 hasAuthorship W2557382017A5077588523 @default.
• W2557382017 hasAuthorship W2557382017A5085708802 @default.
• W2557382017 hasBestOaLocation W25573820171 @default.
• W2557382017 hasConcept C10162356 @default.
• W2557382017 hasConcept C126322002 @default.
• W2557382017 hasConcept C134018914 @default.
• W2557382017 hasConcept C142724271 @default.
• W2557382017 hasConcept C203014093 @default.
• W2557382017 hasConcept C2776225084 @default.
• W2557382017 hasConcept C2776914184 @default.
• W2557382017 hasConcept C2776925932 @default.
• W2557382017 hasConcept C2777636572 @default.
• W2557382017 hasConcept C2778264360 @default.
• W2557382017 hasConcept C2778690821 @default.
• W2557382017 hasConcept C2779134260 @default.
• W2557382017 hasConcept C2779416428 @default.
• W2557382017 hasConcept C2779651940 @default.
• W2557382017 hasConcept C2779969927 @default.
• W2557382017 hasConcept C2910509766 @default.
• W2557382017 hasConcept C2910727661 @default.
• W2557382017 hasConcept C71924100 @default.
• W2557382017 hasConceptScore W2557382017C10162356 @default.
• W2557382017 hasConceptScore W2557382017C126322002 @default.
• W2557382017 hasConceptScore W2557382017C134018914 @default.
• W2557382017 hasConceptScore W2557382017C142724271 @default.
• W2557382017 hasConceptScore W2557382017C203014093 @default.
• W2557382017 hasConceptScore W2557382017C2776225084 @default.
• W2557382017 hasConceptScore W2557382017C2776914184 @default.
• W2557382017 hasConceptScore W2557382017C2776925932 @default.

• next