(Q24313364)

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Defective glycosylation of decorin and biglycan, altered collagen structure, and abnormal phenotype of the skin fibroblasts of an Ehlers-Danlos syndrome patient carrying the novel Arg270Cys substitution in galactosyltransferase I (beta4GalT-7)

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Defective glycosylation of decorin and biglycan, altered collagen structure, and abnormal phenotype of the skin fibroblasts of an Ehlers-Danlos syndrome patient carrying the novel Arg270Cys substitution in galactosyltransferase I (beta4GalT-7) (English)

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Ehlers-Danlos syndrome

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based on heuristic

inferred from title

Beta-1,4-galactosyltransferase 7

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GOA release 2020-03-11

supramolecular fiber organization

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GOA release 2020-03-11

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based on heuristic

inferred from title

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based on heuristic

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3

object named as

Uwe Hansen

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author name string

Daniela G Seidler

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Muhammad Faiyaz-Ul-Haque

2

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George W Yip

4

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Syed H E Zaidi

5

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Ahmad S Teebi

6

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Ludwig Kiesel

7

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Martin Götte

8

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language of work or name

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publication date

July 2006

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Journal of Molecular Medicine

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84

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7

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583-94

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On-line sheathless capillary electrophoresis/nanoelectrospray ionization-tandem mass spectrometry for the analysis of glycosaminoglycan oligosaccharides

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Conformer populations of L-iduronic acid residues in glycosaminoglycan sequences

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Core protein dependence of epimerization of glucuronosyl residues in galactosaminoglycans

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Interaction of biglycan with type I collagen

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A physiologic three-dimensional cell culture system to investigate the role of decorin in matrix organisation and cell survival.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A genetic defect in the biosynthesis of dermatan sulfate proteoglycan: galactosyltransferase I deficiency in fibroblasts from a patient with a progeroid syndrome

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Decorin, a novel player in the insulin-like growth factor system.

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Excess biglycan causes eyelid malformation by perturbing muscle development and TGF-alpha signaling

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cloning and expression of a proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I. A seventh member of the human beta4-galactosyltransferase gene family

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Matrix proteoglycans: from molecular design to cellular function

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structural investigation of chondroitin/dermatan sulfate oligosaccharides from human skin fibroblast decorin

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Decreased expression of lysyl hydroxylase 2 (LH2) in skin fibroblasts from three Ehlers-Danlos patients does not result from mutations in either the coding or proximal promoter region of the LH2 gene.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB

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https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition by the soluble syndecan-1 ectodomains delays wound repair in mice overexpressing syndecan-1.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structural and sequence motifs in dermatan sulfate for promoting fibroblast growth factor-2 (FGF-2) and FGF-7 activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dermatan sulfate proteoglycan and glycosaminoglycan synthesis is induced in fibroblasts by transfer to a three-dimensional extracellular environment

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Deficiency of the decorin core protein in the variant form of Ehlers-Danlos syndrome with chronic skin ulcer

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Different usage of the glycosaminoglycan attachment sites of biglycan

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The matrix component biglycan is proinflammatory and signals through Toll-like receptors 4 and 2 in macrophages

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Chondroitin sulfate in palatal wound healing

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ultrastructure of skin from a patient with mucopolysaccharidosis IIID.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of myoblast migration via decorin expression is critical for normal skeletal muscle differentiation.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Phenotypic effects of biglycan deficiency are linked to collagen fibril abnormalities, are synergized by decorin deficiency, and mimic Ehlers-Danlos-like changes in bone and other connective tissues.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Differential interactions of decorin and decorin mutants with type I and type VI collagens

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A novel missense mutation in the galactosyltransferase-I (B4GALT7) gene in a family exhibiting facioskeletal anomalies and Ehlers-Danlos syndrome resembling the progeroid type

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Deficiency of the core proteins of dermatan sulphate proteoglycans in a variant form of Ehlers-Danlos syndrome

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Defective glycosaminoglycan substitution of decorin in a patient with progeroid syndrome is a direct consequence of two point mutations in the galactosyltransferase I (beta4GalT-7) gene.

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Estimation of the binding force of the collagen molecule-decorin core protein complex in collagen fibril

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The primary structure of the core protein of the small, leucine-rich proteoglycan (PG I) from bovine articular cartilage

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Discrete integration of collagen XVI into tissue-specific collagen fibrils or beaded microfibrils

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular basis for the progeroid variant of Ehlers-Danlos syndrome. Identification and characterization of two mutations in galactosyltransferase I gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glycosaminoglycan-free small proteoglycan core protein is secreted by fibroblasts from a patient with a syndrome resembling progeroid

1 reference

https://pubmed.ncbi.nlm.nih.gov/16583246

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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