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3'-phosphoadenylylsulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
incorporates sulfate exclusively into GalNAc C-6 position
-
-
?
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
24% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Gal(4S)beta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Gal(4S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
270% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
260% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->3)GalNAc(6S)beta(1->4)GlcUAbeta(1->3)Gal-Gal(6S)beta(1->4)Xyl-ol
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->3)GalNAc(6S)beta(1->4)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xyl-ol
360% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->3)GalNAc(6S)beta(1->4)GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl(2P)-ol
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->3)GalNAc(6S)beta(1->4)GlcUAbeta(1->3)Gal(6S)beta(1->3)-Galbeta(1->4)Xyl(2P)-ol
290% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + DELTA4HexUAalpha(1->4)GlcNAc(6S)-GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + DELTA4HexUAalpha(1->4)GlcNAc(6S)beta(1->3)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
17% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
4% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GalNAcbeta(1->4)GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GalNAc(6S)beta(1->4)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
84% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GlcNAcalpha(1->4)GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GlcNAcalpha(1->4)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
2% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GlcUAbeta(1->3)Gal(4S)beta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GlcUAbeta(1->3)Gal(4S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
20% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
adenosine 3',5'-bisphosphate + GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
-
?
3'-phosphoadenylyl sulfate + GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
8% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GlcUAbeta(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Gal(4S)beta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GlcUAbeta(1->3)GalNAc(4S)beta(1->4)GlcUAbeta(1->3)Gal(4S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
250% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylyl sulfate + GlcUAbeta(1->3)GalNAcbeta(1->4)GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xylbeta(1->O)-Ser
adenosine 3',5'-bisphosphate + GlcUAbeta(1->3)GalNAc(6S)beta(1->3)GlcUAbeta(1->3)Gal(6S)beta(1->3)Gal(6S)beta(1->4)Xylbeta(1->O)-Ser
130% of the activity with GlcUAbeta(1->3)Galbeta(1->3)Galbeta(1->4)Xyl-(Gly)Ser-(Gly-Glu)
-
-
?
3'-phosphoadenylylsulfate + beta-benzyl N-acetylglucosamine
adenosine 3',5'-bisphosphate + beta-benzyl N-acetylglucosaminesulfate
-
enzyme does not use alpha-or beta-benzyl N-acetylgalactosamine
-
-
?
3'-phosphoadenylylsulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
3'-phosphoadenylylsulfate + chondroitin 4-sulfate
adenosine 3',5'-bisphosphate + chondroitin 4,6-bissulfate
-
-
-
-
?
3'-phosphoadenylylsulfate + chondroitin sulfate A
adenosine 3',5'-bisphosphate + ?
-
6.2% of the activity with chondroitin
-
-
?
3'-phosphoadenylylsulfate + chondroitin sulfate C
adenosine 3',5'-bisphosphate + ?
-
3% of the activity with chondroitin
-
-
?
3'-phosphoadenylylsulfate + corneal keratan sulfate
?
transfers sulfate to position 6 of Gal residues
-
-
?
3'-phosphoadenylylsulfate + desulfated dermatan sulfate
adenosine 3',5'-bisphosphate + ?
-
95% of the activity with chondroitin
-
-
?
3'-phosphoadenylylsulfate + phenyl 2-acetamido-2-deoxy-4-O-sulfonato-D-galactopyranose
adenosine 3',5'-bisphosphate + phenyl 2-acetamido-2-deoxy-4,6-di-O-sulfonato-D-galactopyranose
-
-
-
-
?
additional information
?
-
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
-
?
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
?
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
-
?
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
?
3'-phosphoadenylyl sulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
-
?
3'-phosphoadenylylsulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
-
-
-
-
?
3'-phosphoadenylylsulfate + chondroitin
adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate
incorporates sulfate exclusively into GalNAc C-6 position
-
-
?
additional information
?
-
keratan sulfate and oligosaccharides containing the Galbeta1-4GlcNAc sequence hardly serve as acceptors for C6ST-2
-
-
?
additional information
?
-
-
keratan sulfate and oligosaccharides containing the Galbeta1-4GlcNAc sequence hardly serve as acceptors for C6ST-2
-
-
?
additional information
?
-
overview: sulfation of chondroitin sulfates
-
-
?
additional information
?
-
-
overview: sulfation of chondroitin sulfates
-
-
?
additional information
?
-
-
no substrate: dermatan sulfate. Enzyme mainly transfers sulfate to GalNAc residues located in the L-iduronic acid rich region with little preference for the GalNAc residue neighbouring the GlcA residue
-
-
?
additional information
?
-
the enzyme mediates the sulfation of proteoglycans, particularly chondroitin sulfate, in the extracellular matrix of cartilage
-
-
?
additional information
?
-
-
the enzyme mediates the sulfation of proteoglycans, particularly chondroitin sulfate, in the extracellular matrix of cartilage
-
-
?
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Adenocarcinoma
Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase.
Atherosclerosis
Human chondroitin 6-sulfotransferase: cloning, gene structure, and chromosomal localization.
Brain Injuries
Chondroitin 6-sulphate synthesis is up-regulated in injured CNS, induced by injury-related cytokines and enhanced in axon-growth inhibitory glia.
chondroitin 6-sulfotransferase deficiency
Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis.
chondroitin 6-sulfotransferase deficiency
Phenotypic features of carbohydrate sulfotransferase 3 (CHST3) deficiency in 24 patients: congenital dislocations and vertebral changes as principal diagnostic features.
Clubfoot
A novel CHST3 allele associated with Spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.
Contracture
A novel CHST3 allele associated with Spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.
Contracture
Recurrent c.776T>C mutation in CHST3 with four other novel mutations and a literature review.
Deafness
Spondyloepiphyseal dysplasia, Omani type: further definition of the phenotype.
Dysostoses
Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis.
Dysostoses
Phenotypic features of carbohydrate sulfotransferase 3 (CHST3) deficiency in 24 patients: congenital dislocations and vertebral changes as principal diagnostic features.
Glioma
OASIS regulates chondroitin 6-O-sulfotransferase 1 gene transcription in the injured adult mouse cerebral cortex.
Hearing Loss
A novel CHST3 allele associated with Spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.
Heart Septal Defects, Ventricular
Spondyloepiphyseal dysplasia, Omani type: further definition of the phenotype.
Hypertension
CHST3 and CHST13 polymorphisms as predictors of bosentan-induced liver toxicity in Japanese patients with pulmonary arterial hypertension.
Intervertebral Disc Degeneration
Carbohydrate sulfotransferase 3 (CHST3) overexpression promotes cartilage endplate-derived stem cells (CESCs) to regulate molecular mechanisms related to repair of intervertebral disc degeneration by rat nucleus pulposus.
Intervertebral Disc Degeneration
Lumbar disc degeneration is linked to a carbohydrate sulfotransferase 3 variant.
Joint Dislocations
A novel CHST3 allele associated with Spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.
Joint Dislocations
Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis.
Joint Dislocations
Prenatal homozygosity mapping detects a novel mutation in CHST3 in a fetus with skeletal dysplasia and joint dislocations.
Joint Dislocations
Recurrent c.776T>C mutation in CHST3 with four other novel mutations and a literature review.
Joint Dislocations
Spondyloepiphyseal dysplasia, Omani type: further definition of the phenotype.
Leukemia
Whole exome sequencing detects CHST3 mutation in patient with acute promyelocytic leukemia: A case report.
Leukemia, Promyelocytic, Acute
Whole exome sequencing detects CHST3 mutation in patient with acute promyelocytic leukemia: A case report.
Liver Diseases
Predictive model of bosentan-induced liver toxicity in Japanese patients with pulmonary arterial hypertension.
Neoplasms
Human chondroitin 6-sulfotransferase: cloning, gene structure, and chromosomal localization.
Osteochondrodysplasias
A novel CHST3 allele associated with Spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred.
Osteochondrodysplasias
A novel nonsense mutation in CHST3 in a Turkish patient with spondyloepiphyseal dysplasia, Omani type.
Osteochondrodysplasias
Expanding the clinical spectrum of B4GALT7 deficiency: homozygous p.R270C mutation with founder effect causes Larsen of Reunion Island syndrome.
Osteochondrodysplasias
Omani-type spondyloepiphyseal dysplasia with cardiac involvement caused by a missense mutation in CHST3.
Osteochondrodysplasias
Spondyloepiphyseal dysplasia Omani type: CHST3 mutation spectrum and phenotypes in three Indian families.
Osteochondrodysplasias
Whole exome sequencing detects CHST3 mutation in patient with acute promyelocytic leukemia: A case report.
Paraplegia
A pharmacogenetic study of docetaxel and thalidomide in patients with castration-resistant prostate cancer using the DMET genotyping platform.
Psoriasis
Chondroitin 6-sulfate represses keratinocyte proliferation in mouse skin, which is associated with psoriasis.
Pulmonary Arterial Hypertension
CHST3 and CHST13 polymorphisms as predictors of bosentan-induced liver toxicity in Japanese patients with pulmonary arterial hypertension.
Pulmonary Emphysema
RNA interference targeting carbohydrate sulfotransferase 3 diminishes macrophage accumulation, inhibits MMP-9 expression and promotes lung recovery in murine pulmonary emphysema.
Seizures
Involvement of chondroitin 6-sulfation in temporal lobe epilepsy.
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L259P
naturally occuring mutation of CHST3 involved in enzyme deficiency and related disease
L286P
naturally occuring mutation of gene CHST3 causing the Spondyloepiphyseal dysplasia Omani type, almost inactive mutant, phenotype, overview
L307P
naturally occuring mutation of CHST3 involved in enzyme deficiency and related disease
R222W
naturally occuring mutation of CHST3 involved in enzyme deficiency and related disease
T141M
homozygous missense mutation leading to a reduction of C6ST-1 activity to 24-29% of the wild type enzyme, the naturally occuring missense mutation in exon 3 of gene CHST3 causes Omani-type spondyloepiphyseal dysplasia with cardiac involvement, phenotype with skeletal features, cardiac involvement including mitral, tricuspid and/or aortic regurgitations and type E brachydactyly, overview
R304Q
-
missense mutation found in patients with a distinct form of spondyloepiphyseal dysplasia, SED Omani type, complete loss of activity, resulting in significant reduction of both DELTAHexA-GalNAc(6S) and DELTAHexA(2S)-GalNAc(6S) and elevate levels of DELTAHexA-GalNAc(4S,6S)
R304Q
naturally occuring mutation of gene CHST3 causing the Spondyloepiphyseal dysplasia Omani type, phenotype, overview
additional information
identification of eight naturally occuring CHST3 mutations in six unrelated individuals who presented at birth with congenital joint dislocations with diagnosis of either Larsen syndrome or humero-spinal dysostosis, the patients show reduced enzyme activity and congenital dislocation of the knees, elbow joint dysplasia with subluxation and limited extension, hip dysplasia or dislocation, clubfoot, short stature, and kyphoscoliosis developing in late childhood, genotyping, enzyme mutant phenotypes, overview
additional information
-
identification of eight naturally occuring CHST3 mutations in six unrelated individuals who presented at birth with congenital joint dislocations with diagnosis of either Larsen syndrome or humero-spinal dysostosis, the patients show reduced enzyme activity and congenital dislocation of the knees, elbow joint dysplasia with subluxation and limited extension, hip dysplasia or dislocation, clubfoot, short stature, and kyphoscoliosis developing in late childhood, genotyping, enzyme mutant phenotypes, overview
additional information
enzyme knockout by siRNA
additional information
-
enzyme knockout by siRNA
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Bhakta, S.; Bartes, A.; Bowman, K.G.; Kao, W.M.; Polsky, I.; Lee, J.K.; Cook, B.N.; Bruehl, R.E.; Rosen, S.D.; Bertozzi, C.R.; Hemmerich, S.
Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)
J. Biol. Chem.
275
40226-40234
2000
Homo sapiens, Mus musculus (Q9EP78), Mus musculus
brenda
Fukuta, M.; Kobayashi, Y.; Uchimura, K.; Kimata, K.; Habuchi, O.
Molecular cloning and expression of human chondroitin 6-sulfotransferase
Biochim. Biophys. Acta
1399
57-61
1998
Homo sapiens (Q7LGC8), Homo sapiens
brenda
Kitagawa, H.; Fujita, M.; Ito, N.; Sugahara, K.
Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase
J. Biol. Chem.
275
21075-21080
2000
Homo sapiens (Q9NS84), Homo sapiens
brenda
Tsutsumi, K.; Shimakawa, H.; Kitagawa, H.; Sugahara, K.
Functional expression and genomic structure of human chondroitin 6-sulfotransferase
FEBS Lett.
441
235-241
1998
Homo sapiens (Q7LGC8), Homo sapiens
brenda
Sawada, T.; Fujii, S.; Nakano, H.; Ohtake, S.; Kimata, K.; Habuchi, O.
Synthesis of sulfated phenyl 2-acetamido-2-deoxy-D-galactopyranosides. 4-O-Sulfated phenyl 2-acetamido-2-deoxy-beta-D-galactopyranoside is a competitive acceptor that decreases sulfation of chondroitin sulfate by N-acetylgalactosamine 4-sulfate 6-O-sulfotransferase
Carbohydr. Res.
340
1983-1996
2005
Homo sapiens
brenda
Yamada, T.; Ohtake, S.; Sato, M.; Habuchi, O.
Chondroitin 4-sulphotransferase-1 and chondroitin 6-sulphotransferase-1 are affected differently by uronic acid residues neighbouring the acceptor GalNAc residues
Biochem. J.
384
567-575
2004
Homo sapiens
brenda
Thiele, H.; Sakano, M.; Kitagawa, H.; Sugahara, K.; Rajab, A.; Hoehne, W.; Ritter, H.; Leschik, G.; Nuernberg, P.; Mundlos, S.
Loss of chondroitin 6-O-sulfotransferase-1 function results in severe human chondrodysplasia with progressive spinal involvement
Proc. Natl. Acad. Sci. USA
101
10155-10160
2004
Homo sapiens
brenda
Gruber, H.E.; Ingram, J.; Leslie, K.; Hanley, E.N.
Gene expression of types I, II, and VI collagen, aggrecan, and chondroitin-6-sulfotransferase in the human annulus: in situ hybridization findings
Spine J.
8
810-817
2008
Homo sapiens
brenda
Hermanns, P.; Unger, S.; Rossi, A.; Perez-Aytes, A.; Cortina, H.; Bonafe, L.; Boccone, L.; Setzu, V.; Dutoit, M.; Sangiorgi, L.; Pecora, F.; Reicherter, K.; Nishimura, G.; Spranger, J.; Zabel, B.; Superti-Furga, A.
Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis
Am. J. Hum. Genet.
82
1368-1374
2008
Homo sapiens (Q7LGC8), Homo sapiens
brenda
van Roij, M.H.; Mizumoto, S.; Yamada, S.; Morgan, T.; Tan-Sindhunata, M.B.; Meijers-Heijboer, H.; Verbeke, J.I.; Markie, D.; Sugahara, K.; Robertson, S.P.
Spondyloepiphyseal dysplasia, Omani type: further definition of the phenotype
Am. J. Med. Genet. A
146A
2376-2384
2008
Homo sapiens (Q7LGC8), Homo sapiens
brenda
Tuysuz, B.; Mizumoto, S.; Sugahara, K.; Celebi, A.; Mundlos, S.; Turkmen, S.
Omani-type spondyloepiphyseal dysplasia with cardiac involvement caused by a missense mutation in CHST3
Clin. Genet.
75
375-383
2009
Homo sapiens (Q7LGC8)
brenda
Kitagawa, H.; Tsutsumi, K.; Ikegami-Kuzuhara, A.; Nadanaka, S.; Goto, F.; Ogawa, T.; Sugahara, K.
Sulfation of the galactose residues in the glycosaminoglycan-protein linkage region by recombinant human chondroitin 6-O-sulfotransferase-1
J. Biol. Chem.
283
27438-27443
2008
Homo sapiens (Q7LGC8), Homo sapiens
brenda
Pan, H.; Xue, W.; Zhao, W.; Schachner, M.
Expression and function of chondroitin 4-sulfate and chondroitin 6-sulfate in human glioma
FASEB J.
34
2853-2868
2020
Homo sapiens (Q7LGC8), Homo sapiens
brenda