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Synonyms
chst14, d4st-1, d4st1, dermatan 4-o-sulfotransferase-1, dermatan 4-o-sulfotransferase 1, chst14/d4st1, dermatan 4-o-sulfotransferase, carbohydrate sulfotransferase 14, n-acetylgalactosamine 4-o-sulfotransferase 1, dermatan-4-sulfotransferase-1,
more
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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
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-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-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-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
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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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additional information
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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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additional information
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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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3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetyl-D-galactosamine
adenosine 3',5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetyl-D-galactosamine
3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
additional information
?
-
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-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-acetyl-D-galactosamine
adenosine 3',5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetyl-D-galactosamine
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-
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-
?
3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
-
-
-
?
3'-phospho-5'-adenylyl sulfate + [dermatan]-N-acetylgalactosamine
adenosine 3'-5'-bisphosphate + [dermatan]-4-O-sulfo-N-acetylgalactosamine
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?
additional information
?
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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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?
additional information
?
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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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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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metabolism
During the biosynthesis of chondroitin/dermatan sulfate (CS/DS), a variable fraction of glucuronic acid is converted to iduronic acid through the activities of two epimerases, dermatan sulfate epimerases 1 (DS-epi1) and 2 (DS-epi2). Without association with other enzymes, DS-epi1 activity produces structures that have only a few adjacent iduronic acid units. In vivo, concomitant with epimerization, dermatan 4-O-sulfotransferase 1 (D4ST1) sulfates the GalNAc adjacent to iduronic acid. This sulfation facilitates DSepi1 activity and enables the formation of long blocks of sulfated iduronic acid-containing domains, which can be major components of CS/DS. Concerted action of DS-epi1 and D4ST1. D4ST1 directly interacts with DS-epi1, but not with DS-epi2. The iduronic acid-forming enzymes operate in complexes, similar to other enzymes active in glycosaminoglycan biosynthesis. siRNA-mediated reduction of DS-epi2 in MCF 10a cells results in a marked reduction of the two epimerases and IdoA biosynthesis
physiological function
D4ST-1 is indispensable in the formation of the important functional domains composed of alternating iduronic acid and 4-O-sulfated N-acetylgalactosamine residues (named 4-O-sulfated iduronic acid blocks) in dermatan sulfate and cannot be compensated by other 4-O-sulfotransferases
malfunction
adducted thumb-clubfoot syndrome is genetically homogeneous and is caused by loss-of-function mutations in CHST14 leading to a deficiency of sulfated dermatan in affected tissues
malfunction
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adducted thumb-clubfoot syndrome is caused by homozygous nonsense and missense mutations in CHST14 which encodes N-acetylgalactosamine 4-O-sulfotransferase 1 leading to congenital malformations, contractures of thumbs and feet, and a typical facial appearance
malfunction
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enzyme deficiency results in impaired neuronal differentiation and diminished neural stem cell proliferation both in vitro and in vivo, associated with an upregulation of the expression of epidermal growth factor receptor and fibroblast growth factor receptor 1and changes in distinct subpopulations of radial glial cells
malfunction
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germline ablation of dermatan-4O-sulfotransferase1 reduces regeneration after mouse spinal cord injury
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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
Mitsunaga, C.; Mikami, T.; Mizumoto, S.; Fukuda, J.; Sugahara, K.
Chondroitin sulfate/dermatan sulfate hybrid chains in the development of cerebellum. Spatiotemporal regulation of the expression of critical disulfated disaccharides by specific sulfotransferases
J. Biol. Chem.
281
18942-18952
2006
Mus musculus (Q80V53), Mus musculus
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
Tetsukawa, A.; Nakamura, J.; Fujiwara, S.
Identification of chondroitin/dermatan sulfotransferases in the protochordate, Ciona intestinalis
Comp. Biochem. Physiol. B
157
205-212
2010
Ciona intestinalis
brenda
Bian, S.; Akyuez, N.; Bernreuther, C.; Loers, G.; Laczynska, E.; Jakovcevski, I.; Schachner, M.
Dermatan sulfotransferase Chst14/D4st1, but not chondroitin sulfotransferase Chst11/C4st1, regulates proliferation and neurogenesis of neural progenitor cells
J. Cell Sci.
124
4051-4063
2011
Mus musculus
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
Rost, S.; Akyuez, N.; Martinovic, T.; Huckhagel, T.; Jakovcevski, I.; Schachner, M.
Germline ablation of dermatan-4O-sulfotransferase1 reduces regeneration after mouse spinal cord injury
Neuroscience
312
74-85
2016
Mus musculus
brenda
Habicher, J.; Haitina, T.; Eriksson, I.; Holmborn, K.; Dierker, T.; Ahlberg, P.; Ledin, J.
Chondroitin/dermatan sulfate modification enzymes in zebrafish development
PLoS ONE
10
e0121957
2015
Danio rerio (Q805E5), Danio rerio
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