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Synonyms
chst14, d4st-1, d4st1, dermatan 4-o-sulfotransferase-1, chst14/d4st1, dermatan 4-o-sulfotransferase 1, dermatan 4-o-sulfotransferase, carbohydrate sulfotransferase 14, dermatan-4-sulfotransferase-1, n-acetylgalactosamine 4-o-sulfotransferase 1,
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetyl-D-galactosamine
adenosine 3',5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetyl-D-galactosamine
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
additional information
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
dermatan 4-O-sulfotransferase 1 is pivotal in the formation of iduronic acid blocks in dermatan sulfate
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
desulfated dermatan sulfate from porcine intestine. The sulfation takes place at the 4-position of N-acetylgalactosamine residues of dermatan. D4ST-1 transfers sulfate to the C-4 hydroxyl of (1,4)-linked GalNAc that is substituted with an alpha-linked iduronic acid at the C-3 hydroxyl. D4ST-1 shows a strong preference in vitro for sulfate transfer to IdoUAalpha(1,3)GalNAcbeta(1,4) that is flanked by GlcUAbeta(1,3)GalNAcbeta(1,4) as compared with IdoUAalpha(1,3)GalNAcbeta(1,4) flanked by IdoUAalpha1,3GalNAcbeta1,4. The specificity of D4ST-1 when assayed in vitro suggests that the addition of sulfate to GalNAc occurs immediately after epimerization of GlcUA to IdoUA. While D4ST-1 is able to transfer sulfate to both dermatan and chondroitin, the rate of transfer is nearly 10fold greater to dermatan than to chondroitin when the same substrate concentrations are used. D4ST-1 only displays a significant transfer of sulfate to chondroitin at relatively high substrate concentrations, raising the possibility that the chondroitin is contaminated with small amounts of dermatan and/or that occasional iduronic acid residues are present in the chondroitin and utilized by the D4ST-1
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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
most efficiently utilizes GalNAc residues located not only in the sequence -IdoUA-GalNAc-IdoUA- but also in -GlcUA-Gal-NAc-IdoUA- and -IdoUA-GalNAc-GlcUA-. The isolated oligosaccharide structures suggest that 4-O-sulfation promotes subsequent 4-O-sulfation of GalNAc in the neighboring disaccharide unit
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dermatan sulfate epimerase 1 and dermatan 4-O-sulfotransferase 1 form complexes that generate long epimerized 4-O-sulfated blocks
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the enzyme catalyzes the 4-O-sulfation of N-acetylgalactosamine in the repeating iduronic acid-alpha-1,3-N-acetylgalactosamine disaccharide sequence to form dermatan sulfate
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Clubfoot
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Clubfoot
Musculocontractural Ehlers-Danlos Syndrome (former EDS type VIB) and adducted thumb clubfoot syndrome (ATCS) represent a single clinical entity caused by mutations in the dermatan-4-sulfotransferase 1 encoding CHST14 gene.
Congenital Abnormalities
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Congenital Abnormalities
Defect in dermatan sulfate in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency.
Congenital Disorders of Glycosylation
Congenital disorders of glycosylation with emphasis on loss of dermatan-4-sulfotransferase.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Congenital disorders of glycosylation with emphasis on loss of dermatan-4-sulfotransferase.
Contracture
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Contracture
Genetic heterogeneity and clinical variability in musculocontractural Ehlers-Danlos syndrome caused by impaired dermatan sulfate biosynthesis.
Craniofacial Abnormalities
Genetic heterogeneity and clinical variability in musculocontractural Ehlers-Danlos syndrome caused by impaired dermatan sulfate biosynthesis.
dermatan 4-sulfotransferase deficiency
A 45-year-old Woman with Ehlers-Danlos Syndrome Caused by Dermatan 4-O-sulfotransferase-1 Deficiency: Implications for Early Ageing.
dermatan 4-sulfotransferase deficiency
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
dermatan 4-sulfotransferase deficiency
Defect in dermatan sulfate in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency.
dermatan 4-sulfotransferase deficiency
Delineation of dermatan 4-O-sulfotransferase 1 deficient Ehlers-Danlos syndrome: Observation of two additional patients and comprehensive review of 20 reported patients.
dermatan 4-sulfotransferase deficiency
Impaired Cognitive Function and Altered Hippocampal Synaptic Plasticity in Mice Lacking Dermatan Sulfotransferase Chst14/D4st1.
dermatan 4-sulfotransferase deficiency
Myopathy in a 20-year-old female patient with D4ST-1 deficient Ehlers-Danlos syndrome due to a homozygous CHST14 mutation.
dermatan 4-sulfotransferase deficiency
Spinal manifestations in 12 patients with musculocontractural Ehlers-Danlos syndrome caused by CHST14/D4ST1 deficiency (mcEDS-CHST14).
dermatan 4-sulfotransferase deficiency
Structural alteration of glycosaminoglycan side chains and spatial disorganization of collagen networks in the skin of patients with mcEDS-CHST14.
dermatan 4-sulfotransferase deficiency
Systematic investigation of the skin in Chst14-/- mice: a model for skin fragility in musculocontractural Ehlers-Danlos syndrome caused by CHST14 variants (mcEDS-CHST14).
dermatan 4-sulfotransferase deficiency
Vascular abnormalities in the placenta of Chst14-/- fetuses: implications in the pathophysiology of perinatal lethality of the murine model and vascular lesions in human CHST14/D4ST1 deficiency.
Ehlers-Danlos Syndrome
A 45-year-old Woman with Ehlers-Danlos Syndrome Caused by Dermatan 4-O-sulfotransferase-1 Deficiency: Implications for Early Ageing.
Ehlers-Danlos Syndrome
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome
Defect in dermatan sulfate in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency.
Ehlers-Danlos Syndrome
Delineation of dermatan 4-O-sulfotransferase 1 deficient Ehlers-Danlos syndrome: Observation of two additional patients and comprehensive review of 20 reported patients.
Ehlers-Danlos Syndrome
Delineation of musculocontractural Ehlers-Danlos Syndrome caused by dermatan sulfate epimerase deficiency.
Ehlers-Danlos Syndrome
Dermatan 4-O-sulfotransferase 1-deficient Ehlers-Danlos syndrome complicated by a large subcutaneous hematoma on the back.
Ehlers-Danlos Syndrome
Extracellular matrix and platelet function in patients with musculocontractural Ehlers-Danlos syndrome caused by mutations in the CHST14 gene.
Ehlers-Danlos Syndrome
Loss of dermatan sulfate epimerase (DSE) function results in musculocontractural Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome
Loss-of-function mutations of CHST14 in a new type of Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome
Musculocontractural Ehlers-Danlos Syndrome (former EDS type VIB) and adducted thumb clubfoot syndrome (ATCS) represent a single clinical entity caused by mutations in the dermatan-4-sulfotransferase 1 encoding CHST14 gene.
Ehlers-Danlos Syndrome
Myopathy in a 20-year-old female patient with D4ST-1 deficient Ehlers-Danlos syndrome due to a homozygous CHST14 mutation.
Ehlers-Danlos Syndrome
Novel mutation in the CHST14 gene causing musculocontractural type of Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome
Posterior Spinal Fusion for Severe Spinal Deformities in Musculocontractural Ehlers-Danlos Syndrome: Detailed Observation of a Novel Case and Review of 2 Reported Cases.
Ehlers-Danlos Syndrome
Re-assigned diagnosis of D4ST1-deficient Ehlers-Danlos syndrome (adducted thumb-clubfoot syndrome) after initial diagnosis of Marden-Walker syndrome.
Ehlers-Danlos Syndrome
Spinal manifestations in 12 patients with musculocontractural Ehlers-Danlos syndrome caused by CHST14/D4ST1 deficiency (mcEDS-CHST14).
Ehlers-Danlos Syndrome
Structural alteration of glycosaminoglycan side chains and spatial disorganization of collagen networks in the skin of patients with mcEDS-CHST14.
Ehlers-Danlos Syndrome
Systematic investigation of the skin in Chst14-/- mice: a model for skin fragility in musculocontractural Ehlers-Danlos syndrome caused by CHST14 variants (mcEDS-CHST14).
Ehlers-Danlos Syndrome
The phenotype of the musculocontractural type of Ehlers-Danlos syndrome due to CHST14 mutations.
Hypertelorism
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Muscle Weakness
Myopathy in a 20-year-old female patient with D4ST-1 deficient Ehlers-Danlos syndrome due to a homozygous CHST14 mutation.
Muscular Diseases
Myopathy in a 20-year-old female patient with D4ST-1 deficient Ehlers-Danlos syndrome due to a homozygous CHST14 mutation.
Pneumothorax
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
procollagen-lysine 5-dioxygenase deficiency
Delineation of dermatan 4-O-sulfotransferase 1 deficient Ehlers-Danlos syndrome: Observation of two additional patients and comprehensive review of 20 reported patients.
Spinal Cord Injuries
Germline ablation of dermatan-4O-sulfotransferase1 reduces regeneration after mouse spinal cord injury.
Spinal Cord Injuries
Knockdown of chondroitin-4-sulfotransferase-1, but not of dermatan-4-sulfotransferase-1, accelerates regeneration of zebrafish after spinal cord injury.
Stomach Neoplasms
Pan-Cancer Analysis Reveals Distinct Metabolic Reprogramming in Different Epithelial-Mesenchymal Transition Activity States.
Talipes
CHST14/D4ST1 deficiency: New form of Ehlers-Danlos syndrome.
Talipes
Defect in dermatan sulfate in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency.
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Mikami, T.; Mizumoto, S.; Kago, N.; Kitagawa, H.; Sugahara, K.
Specificities of three distinct human chondroitin/dermatan N-acetylgalactosamine 4-O-sulfotransferases demonstrated using partially desulfated dermatan sulfate as an acceptor. Implication of differential roles in dermatan sulfate biosynthesis
J. Biol. Chem.
278
36115-36127
2003
Homo sapiens (Q8NCH0), Homo sapiens
brenda
Tiedemann, K.; Olander, B.; Eklund, E.; Todorova, L.; Bengtsson, M.; Maccarana, M.; Westergren-Thorsson, G.; Malmstroem, A.
Regulation of the chondroitin/dermatan fine structure by transforming growth factor-b1 through effects on polymer-modifying enzymes
Glycobiology
15
1277-1285
2005
Homo sapiens (Q8NCH0), Homo sapiens
brenda
Dndar, M.; Mller, T.; Zhang, Q.; Pan, J.; Steinmann, B.; Vodopiutz, J.; Gruber, R.; Sonoda, T.; Krabichler, B.; Utermann, G.; Baenziger, J.U.; Zhang, L.; Janecke, A.R.
Loss of dermatan-4-sulfotransferase 1 function results in adducted thumb-clubfoot syndrome
Am. J. Hum. Genet.
85
873-882
2009
Homo sapiens (Q8NCH0), Homo sapiens
brenda
Pacheco, B.; Maccarana, M.; Malmstrm, A.
Dermatan 4-O-sulfotransferase 1 is pivotal in the formation of iduronic acid blocks in dermatan sulfate
Glycobiology
19
1197-1203
2009
Homo sapiens (Q8NCH0), Homo sapiens
brenda
Evers, M.R.; Xia, G.; Kang, H.G.; Schachner, M.; Baenziger, J.U.
Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase
J. Biol. Chem.
276
36344-36353
2001
Homo sapiens (Q8NCH0), Homo sapiens
brenda
Zhang, L.; Mueller, T.; Baenziger, J.U.; Janecke, A.R.
Congenital disorders of glycosylation with emphasis on loss of dermatan-4-sulfotransferase
Prog. Mol. Biol. Transl. Sci.
93
289-307
2010
Homo sapiens
brenda
Tykesson, E.; Hassinen, A.; Zielinska, K.; Thelin, M.A.; Frati, G.; Ellervik, U.; Westergren-Thorsson, G.; Malmstroem, A.; Kellokumpu, S.; Maccarana, M.
Dermatan sulfate epimerase 1 and dermatan 4-O-sulfotransferase 1 form complexes that generate long epimerized 4-O-sulfated blocks
J. Biol. Chem.
293
13725-13735
2018
Homo sapiens (Q8NCH0)
brenda