Gene Symbol: CSGALNACT1
Description: chondroitin sulfate N-acetylgalactosaminyltransferase 1
Alias: CSGalNAcT-1, ChGn, ChGn-1, beta4GalNAcT, chondroitin sulfate N-acetylgalactosaminyltransferase 1, beta4GalNAcT-1, chondroitin beta-1,4-N-acetylgalactosaminyltransferase 1, chondroitin beta1,4 N-acetylgalactosaminyltransferase, glucuronylgalactosylproteoglycan 4-beta-N- acetylgalactosaminyltransferase
Species: human
Products:     CSGALNACT1

Top Publications

  1. Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, et al. Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate. J Biol Chem. 2003;278:3063-71 pubmed
    ..Quantitative real-time PCR analysis revealed that CSGalNAcT-2 transcripts were highly expressed in the small intestine, leukocytes, and spleen, however, both CSGalNAcTs were ubiquitously expressed in various tissues. ..
  2. Watanabe Y, Takeuchi K, Higa Onaga S, Sato M, Tsujita M, Abe M, et al. Chondroitin sulfate N-acetylgalactosaminyltransferase-1 is required for normal cartilage development. Biochem J. 2010;432:47-55 pubmed publisher
    ..To understand the physiological roles of enzymes involved in CS synthesis, we produced CSGalNAcT1 (CS N-acetylgalactosaminyltransferase 1)-null mice...
  3. Kitagawa H, Uyama T, Sugahara K. Molecular cloning and expression of a human chondroitin synthase. J Biol Chem. 2001;276:38721-6 pubmed
    ..Thus, we demonstrated that analogous to human heparan sulfate polymerases, the single polypeptide chondroitin synthase possesses two glycosyltransferase activities required for chain polymerization. ..
  4. Agnelli L, Forcato M, Ferrari F, Tuana G, Todoerti K, Walker B, et al. The reconstruction of transcriptional networks reveals critical genes with implications for clinical outcome of multiple myeloma. Clin Cancer Res. 2011;17:7402-12 pubmed publisher
    ..model, comprising FAM53B, KIF21B, WHSC1, and TMPO, and a neighbor-gene model, comprising BLNK shared neighbors CSGALNACT1 and SLC7A7, predicted survival in all data sets with follow-up information...
  5. Uyama T, Kitagawa H, Tamura Ji J, Sugahara K. Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfat. J Biol Chem. 2002;277:8841-6 pubmed
    ..Thus, more than two distinct enzymes forming the novel gene family are required for chain initiation and elongation in chondroitin/dermatan sulfate as in the biosynthesis of heparin/heparan sulfate. ..
  6. Sato T, Kudo T, Ikehara Y, Ogawa H, Hirano T, Kiyohara K, et al. Chondroitin sulfate N-acetylgalactosaminyltransferase 1 is necessary for normal endochondral ossification and aggrecan metabolism. J Biol Chem. 2011;286:5803-12 pubmed publisher
    ..b>Csgalnact1(-/-) mice were viable and fertile but exhibited slight dwarfism...
  7. Sakai K, Kimata K, Sato T, Gotoh M, Narimatsu H, Shinomiya K, et al. Chondroitin sulfate N-acetylgalactosaminyltransferase-1 plays a critical role in chondroitin sulfate synthesis in cartilage. J Biol Chem. 2007;282:4152-61 pubmed
    ..Our studies may lead to a new therapeutic intervention, ameliorating the outcome of cartilage degenerative diseases. ..
  8. Adhikara I, Yagi K, Mayasari D, Ikeda K, Kitagawa H, Miyata O, et al. Chondroitin sulfate N-acetylgalactosaminyltransferase-2 deletion alleviates lipoprotein retention in early atherosclerosis and attenuates aortic smooth muscle cell migration. Biochem Biophys Res Commun. 2018;: pubmed publisher
    ..Chondroitin sulfate N-acetylgalactosaminyltransferase-2 (ChGn-2) is a vital Golgi transferase that participates in enzymatic elongation of GAGs...
  9. Yamada J, Nadanaka S, Kitagawa H, Takeuchi K, Jinno S. Increased synthesis of chondroitin sulfate proteoglycan promotes adult hippocampal neurogenesis in response to enriched environment. J Neurosci. 2018;: pubmed publisher
    ..granule cells were also decreased in mice that lacked chondroitin sulfate N-acetylgalactosaminyltransferase 1 (CSGalNAcT1), a key enzyme for CSPG synthesis (T1KO mice)...

More Information


  1. Izumikawa T, Sato B, Mikami T, Tamura J, Igarashi M, Kitagawa H. GlcUAβ1-3Galβ1-3Galβ1-4Xyl(2-O-phosphate) is the preferred substrate for chondroitin N-acetylgalactosaminyltransferase-1. J Biol Chem. 2015;290:5438-48 pubmed publisher
    A deficiency in chondroitin N-acetylgalactosaminyltransferase-1 (ChGn-1) was previously shown to reduce the number of chondroitin sulfate (CS) chains, leading to skeletal dysplasias in mice, suggesting that ChGn-1 regulates the number of ..
  2. Shimbo M, Suzuki R, Fuseya S, Sato T, Kiyohara K, Hagiwara K, et al. Postnatal lethality and chondrodysplasia in mice lacking both chondroitin sulfate N-acetylgalactosaminyltransferase-1 and -2. PLoS ONE. 2017;12:e0190333 pubmed publisher
    ..Previous studies have suggested that two glycosyltransferases, Csgalnact1 (t1) and Csgalnact2 (t2), are critical for initiation of CS synthesis in vitro...
  3. Grzegorzewska A, Ostromecki G, Zielińska P, Mostowska A, Niemir Z, Polcyn Adamczak M, et al. Association of Retinoid X Receptor Alpha Gene Polymorphism with Clinical Course of Chronic Glomerulonephritis. Med Sci Monit. 2015;21:3671-81 pubmed
    ..VD binding protein, VD receptor (VDR), and retinoids are involved in pathogenesis of chronic glomerulonephritis (ChGN)...
  4. Bret C, Hose D, Reme T, Sprynski A, Mahtouk K, Schved J, et al. Expression of genes encoding for proteins involved in heparan sulphate and chondroitin sulphate chain synthesis and modification in normal and malignant plasma cells. Br J Haematol. 2009;145:350-68 pubmed publisher
    ..genes were significantly different between normal and malignant plasma cells, nine of these genes -EXT2, CHSY3, CSGALNACT1, HS3ST2, HS2ST1, CHST11, CSGALNACT2, HPSE, SULF2 - encode proteins involved in glycosaminoglycan chain synthesis ..
  5. Zheng J, Wu C, Ma W, Zhang Y, Hou T, Xu H, et al. Abnormal expression of chondroitin sulphate N-acetylgalactosaminyltransferase 1 and Hapln-1 in cartilage with Kashin-Beck disease and primary osteoarthritis. Int Orthop. 2013;37:2051-9 pubmed publisher
    ..It was indicated that CSGalNAcT-1 and Hapln-1 may play important roles in the pathogenesis of KBD and OA. ..
  6. Ishimaru D, Sugiura N, Akiyama H, Watanabe H, Matsumoto K. Alterations in the chondroitin sulfate chain in human osteoarthritic cartilage of the knee. Osteoarthritis Cartilage. 2014;22:250-8 pubmed publisher
    ..021; chain length: 5.36 vs 6.19 kDa, P = 0.026). Three genes encoding CS glycosyltransferases (CHPF, CSGALNACT1, CSGALNACT2) were expressed at lower levels in lesion cartilage...
  7. Hou X, Yoshioka N, Tsukano H, Sakai A, Miyata S, Watanabe Y, et al. Chondroitin Sulfate Is Required for Onset and Offset of Critical Period Plasticity in Visual Cortex. Sci Rep. 2017;7:12646 pubmed publisher
    ..of ocular dominance plasticity was impaired with reduced CS, using mice lacking a key CS-synthesizing enzyme, CSGalNAcT1. Two-photon in vivo imaging showed a weaker visual response of PV-cells with reduced CS compared to wild-type ..
  8. Schulten H, Al Mansouri Z, Baghallab I, Bagatian N, Subhi O, Karim S, et al. Comparison of microarray expression profiles between follicular variant of papillary thyroid carcinomas and follicular adenomas of the thyroid. BMC Genomics. 2015;16 Suppl 1:S7 pubmed publisher
    ..receptor 155 (GPR155), Purkinje cell protein 4 (PCP4), chondroitin sulfate N-acetylgalactosaminyltransferase 1 (CSGALNACT1), and glutamate receptor interacting protein 1 (GRIP1)...
  9. Iida J, Dorchak J, Clancy R, Slavik J, Ellsworth R, Katagiri Y, et al. Role for chondroitin sulfate glycosaminoglycan in NEDD9-mediated breast cancer cell growth. Exp Cell Res. 2015;330:358-70 pubmed publisher
    ..Importantly, enzymes generating chondroitin sulfate glycosaminoglycans (CS) such as CHST11, CHST15, and CSGALNACT1 were upregulated in HCC38(NEDD9) compared to HCC38(Vector)...
  10. Yoshioka N, Miyata S, Tamada A, Watanabe Y, Kawasaki A, Kitagawa H, et al. Abnormalities in perineuronal nets and behavior in mice lacking CSGalNAcT1, a key enzyme in chondroitin sulfate synthesis. Mol Brain. 2017;10:47 pubmed publisher
    ..the importance of CS, we produced and precisely examined mice that were deficient in the CS synthesizing enzyme, CSGalNAcT1 (T1KO)...
  11. Gelernter J, Kranzler H, Sherva R, Almasy L, Herman A, Koesterer R, et al. Genome-wide association study of nicotine dependence in American populations: identification of novel risk loci in both African-Americans and European-Americans. Biol Psychiatry. 2015;77:493-503 pubmed publisher
    ..10 × 10(-9) at rs6996964 (adjusted for other substances), located between CSGALNACT1 and INTS10...
  12. Saigoh K, Izumikawa T, Koike T, Shimizu J, Kitagawa H, Kusunoki S. Chondroitin beta-1,4-N-acetylgalactosaminyltransferase-1 missense mutations are associated with neuropathies. J Hum Genet. 2011;56:143-6 pubmed publisher
    ..We investigated the chondroitin beta1,4-N-acetylgalactosaminyltransferase-1 (ChGn-1) gene in 114 patients affected with neuropathies including Guillain-Barré syndrome, chronic inflammatory ..
  13. Igarashi M, Takeuchi K, Sugiyama S. Roles of CSGalNAcT1, a key enzyme in regulation of CS synthesis, in neuronal regeneration and plasticity. Neurochem Int. 2018;119:77-83 pubmed publisher
    ..Here we focus on one of those enzymes, CSGalNAcT1 (T1)...
  14. Gulberti S, Jacquinet J, Chabel M, Ramalanjaona N, Magdalou J, Netter P, et al. Chondroitin sulfate N-acetylgalactosaminyltransferase-1 (CSGalNAcT-1) involved in chondroitin sulfate initiation: Impact of sulfation on activity and specificity. Glycobiology. 2012;22:561-71 pubmed publisher
    ..Our results strongly suggest that sulfation of the linkage region acts as a regulatory signal in CS chain initiation. ..
  15. Izumikawa T, Saigoh K, Shimizu J, Tsuji S, Kusunoki S, Kitagawa H. A chondroitin synthase-1 (ChSy-1) missense mutation in a patient with neuropathy impairs the elongation of chondroitin sulfate chains initiated by chondroitin N-acetylgalactosaminyltransferase-1. Biochim Biophys Acta. 2013;1830:4806-12 pubmed publisher
  16. Coolen N, Schouten K, Middelkoop E, Ulrich M. Comparison between human fetal and adult skin. Arch Dermatol Res. 2010;302:47-55 pubmed publisher
    ..This study suggests that, especially, dermal components are important in fetal scarless healing...
  17. Ritelli M, Chiarelli N, Zoppi N, Dordoni C, Quinzani S, Traversa M, et al. Insights in the etiopathology of galactosyltransferase II (GalT-II) deficiency from transcriptome-wide expression profiling of skin fibroblasts of two sisters with compound heterozygosity for two novel B3GALT6 mutations. Mol Genet Metab Rep. 2015;2:1-15 pubmed publisher
    ..i>COL5A1, and COL15A1, enzymes involved in GAG synthesis and in ECM remodeling, such as CSGALNACT1, CHPF, LOXL3, and STEAP4, signaling transduction molecules of the TGF?/BMP pathway, i.e...
  18. Clausen T, Pereira M, Al Nakouzi N, Oo H, Agerbæk M, Lee S, et al. Oncofetal Chondroitin Sulfate Glycosaminoglycans Are Key Players in Integrin Signaling and Tumor Cell Motility. Mol Cancer Res. 2016;14:1288-1299 pubmed
    ..enzymes β-1,3-glucuronyltransferase 1 (B3GAT1) and chondroitin sulfate N-acetylgalactosaminyltransferase 1 (CSGALNACT1)...
  19. Kesselmeier M, Pütter C, Volckmar A, Baurecht H, Grallert H, Illig T, et al. High-throughput DNA methylation analysis in anorexia nervosa confirms TNXB hypermethylation. World J Biol Psychiatry. 2018;19:187-199 pubmed publisher
    ..For a single nucleotide polymorphism rs923768 in CSGALNACT1 a nearby site was nominally associated with AN. At the gene level, we confirmed hypermethylated sites at TNXB...
  20. Vodopiutz J, Mizumoto S, Lausch E, Rossi A, Unger S, Janocha N, et al. Chondroitin Sulfate N-acetylgalactosaminyltransferase-1 (CSGalNAcT-1) Deficiency Results in a Mild Skeletal Dysplasia and Joint Laxity. Hum Mutat. 2017;38:34-38 pubmed publisher
    ..Pro384Arg missense mutation in CSGALNACT1 was found...
  21. Yong Z, Zaini Z, Kallarakkal T, Karen Ng L, Rahman Z, Ismail S, et al. Genetic alterations of chromosome 8 genes in oral cancer. Sci Rep. 2014;4:6073 pubmed publisher
    ..The most frequent gene losses found were the GATA4 gene (24.2%), FGFR1 gene (24.2%), MSRA (21.2) and CSGALNACT1 (12.1%)...
  22. Munkley J. Glycosylation is a global target for androgen control in prostate cancer cells. Endocr Relat Cancer. 2017;24:R49-R64 pubmed publisher
    ..cancer cells and further defined a set of 8 glycosylation enzymes (GALNT7, ST6GalNAc1, GCNT1, UAP1, PGM3, CSGALNACT1, ST6GAL1 and EDEM3), which are also significantly upregulated in prostate cancer tissue...
  23. Saigoh K, Yoshimura S, Izumikawa T, Miyata S, Tabara Y, Matsushita T, et al. Chondroitin sulfate β-1,4-N-acetylgalactosaminyltransferase-1 (ChGn-1) polymorphism: Association with progression of multiple sclerosis. Neurosci Res. 2016;108:55-9 pubmed publisher
    ..b>ChGn-1 is a key enzyme for production of CSPGs and knock-out mice of this gene showed better recovery from spinal cord ..
  24. Munkley J, Vodák D, Livermore K, James K, Wilson B, Knight B, et al. Glycosylation is an Androgen-Regulated Process Essential for Prostate Cancer Cell Viability. EBioMedicine. 2016;8:103-116 pubmed publisher
    ..Of 31 reciprocally-regulated glycosylation enzymes, a set of 8 (GALNT7, ST6GalNAc1, GCNT1, UAP1, PGM3, CSGALNACT1, ST6GAL1 and EDEM3) were significantly up-regulated in clinical prostate carcinoma...
  25. Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, et al. Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis. J Biol Chem. 2002;277:38189-96 pubmed
    ..This is the first report of a chondroitin pentasaccharide constructed with recombinant glycosyltransferases in vitro. ..
  26. Hu B, Xu C, Tian Y, Shi C, Zhang Y, Deng L, et al. Inflammatory microRNA-194 and -515 attenuate the biosynthesis of chondroitin sulfate during human intervertebral disc degeneration. Oncotarget. 2017;8:49303-49317 pubmed publisher
  27. Hunter A, Leuchter A, Power R, Muthen B, McGrath P, Lewis C, et al. A genome-wide association study of a sustained pattern of antidepressant response. J Psychiatr Res. 2013;47:1157-65 pubmed publisher
    ..11×10(-7)), which resides in chondroitin sulfate N-acetylgalactosaminyltransferase 1 gene (CSGALNACT1)...
  28. Anggraeni V, Emoto N, Yagi K, Mayasari D, Nakayama K, Izumikawa T, et al. Correlation of C4ST-1 and ChGn-2 expression with chondroitin sulfate chain elongation in atherosclerosis. Biochem Biophys Res Commun. 2011;406:36-41 pubmed publisher
    ..2 Golgi enzymes, chondroitin 4-O-sulfotransferase-1 (C4ST-1) and chondroitin N-acetylgalactosaminyltransferase-2 (ChGn-2), play a critical role in CS chain elongation...
  29. Duchez S, Pascal V, Cogne N, Jayat Vignoles C, Julien R, Cogne M. Glycotranscriptome study reveals an enzymatic switch modulating glycosaminoglycan synthesis during B-cell development and activation. Eur J Immunol. 2011;41:3632-44 pubmed publisher
    ..Together the data suggest that despite their low amounts in lymphocytes, the heparan sulfate chains decorating the endogenous GAGs appear to be regulators of B-cell physiology. ..
  30. Mikami T, Kitagawa H. Biosynthesis and function of chondroitin sulfate. Biochim Biophys Acta. 2013;1830:4719-33 pubmed publisher
    ..Control of enzymes responsible for CS biosynthesis/catabolism is a potential target for therapeutic intervention for the CS-associated disorders. ..