2.4.1.80: ceramide glucosyltransferase
This is an abbreviated version!
For detailed information about ceramide glucosyltransferase, go to the full flat file.
Word Map on EC 2.4.1.80
-
2.4.1.80
-
glucosylceramide
-
glycosphingolipids
-
sphingolipids
-
gangliosides
-
gm3
-
sphingoid
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galactosylceramide
-
d-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol
-
gaucher
-
d-pdmp
-
n-butyldeoxynojirimycin
-
nb-dnj
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1-phenyl-2-decanoylamino-3-morpholino-1-propanol
-
galcers
-
medicine
-
udp-galactose:ceramide
-
glucocerebroside
-
cgalt
-
laccer
-
conduritol
-
pharmacology
-
molecular biology
-
drug development
- 2.4.1.80
- glucosylceramide
- glycosphingolipids
- sphingolipids
- gangliosides
- gm3
-
sphingoid
- galactosylceramide
- d-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol
- gaucher
-
d-pdmp
- n-butyldeoxynojirimycin
-
nb-dnj
- 1-phenyl-2-decanoylamino-3-morpholino-1-propanol
-
galcers
- medicine
-
udp-galactose:ceramide
- glucocerebroside
- cgalt
-
laccer
-
conduritol
- pharmacology
- molecular biology
- drug development
Reaction
Synonyms
ceramide glucosyltransferase, ceramide glycosyltransferase, ceramide:UDP-glucose glucosyltransferase, ceramide:UDPGlc glucosyltransferase, cerebroside synthase, CGT, cgt-1, cgt-2, cgt-3, GCS, GCS1, GlcCer synthase, GlcCerS, GlcT, GlcT-1, glucosylceramide synthase, glucosyltransferase, uridine diphosphoglucose-ceramide, GluT, UDP glucose-ceramide glucosyltransferase, UDP-Glc:ceramide glucosyltransferase, UDP-glucoase:ceramide glucosyltransferase, UDP-glucose ceramide glucosyltransferase, UDP-glucose ceramide glycosyltransferase, UDP-glucose: ceramide glucosyltransferase, UDP-glucose:ceramide glucosyltransferase, Ugcg, uridine diphosphate (UDP)-glucose:N-acylsphingosine D-glucosyltransferase, uridine diphosphoglucose-ceramide glucosyltransferase
ECTree
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Reference on EC 2.4.1.80 - ceramide glucosyltransferase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Paul, P.; Lutz, T.M.; Osborn, C.; Kyosseva, S.; Elbein, A.D.; Towbin, H.; Radominska, A.; Drake, R.R.
Synthesis and characterization of a new class of inhibitors of membrane-associated UDP-glycosyltransferases
J. Biol. Chem.
268
12933-12938
1993
Mus musculus
Basu, S.; Kaufman, B.; Roseman, S.
Enzymatic synthesis of glucocerebroside by a glucosyltransferase from embryonic chicken brain
J. Biol. Chem.
248
1388-1394
1973
Cavia porcellus, Gallus gallus, Ovis aries, Homo sapiens, Rattus norvegicus, Sus scrofa
Shah, S.N.
UDP-glucose: ceramide glucosyltransferase of rat brain: an association with smooth microsomes and requirement of an intact membrane for enzyme activity
Arch. Biochem. Biophys.
159
143-150
1973
Rattus norvegicus, Rattus norvegicus Sprague-Dawley
Shukla, G.S.; Radin, N.S.
Glucosyceramide synthase of mouse kidney: further characterization with an improved assay method
Arch. Biochem. Biophys.
283
372-378
1990
Mus musculus
Matsuo, N.; Nomura, T.; Imokawa, G.
A rapid and simple assay method for UDP-glucose:ceramide glucosyltransferase
Biochim. Biophys. Acta
1116
97-103
1992
Rattus norvegicus
Vunnam, R.R.; Radin, N.S.
Short chain ceramides as substrates for glucocerebroside synthetase. Differences between liver and brain enzymes
Biochim. Biophys. Acta
573
73-82
1979
Mus musculus
Durieux, I.; Martel, M.B.; Got, R.
Solubilization of UDPglucose-ceramide glucosyltransferase from the Golgi apparatus
Biochim. Biophys. Acta
1024
263-266
1990
Sus scrofa
Coste, H.; Martel, M.B.; Azzar, G.; Got, R.
UDPglucose-ceramide glucosyltransferase from porcine submaxillary glands is associated with the Golgi apparatus
Biochim. Biophys. Acta
814
1-7
1985
Sus scrofa
Nakayama, M.; Kojima, M.; Ohnishi, M.; Ito, S.
Enzymatic formation of plant cerebroside: properties of UDP-glucose:ceramide glucosyltransferase in radish seedlings
Biosci. Biotechnol. Biochem.
59
1882-1886
1995
Raphanus sativus
Ichikawa, S.; Sakiyama, H.; Suzuki, G.; Hidari, K.I.P.J.; Hirabayashi, Y.
Expression cloning of a cDNA for human ceramide glucosyltransferase that catalyzes the first glycosylation step of glycosphingolipid synthesis
Proc. Natl. Acad. Sci. USA
93
4638-4643
1996
Homo sapiens (Q16739), Homo sapiens
Paul, P.; Kamisaka, Y.; Marks, D.L.; Pagano, R.E.
Purification and characterization of UDP-glucose:ceramide glucosyltransferase from rat liver Golgi membranes
J. Biol. Chem.
271
2287-2293
1996
Rattus norvegicus
Chujor, C.S.; Feingold, K.R.; Elias, P.M.; Holleran, W.M.
Glucosylceramide synthase activity in murine epidermis: quantitation, localization, regulation, and requirement for barrier homeostasis
J. Lipid Res.
39
277-285
1998
Mus musculus
Ichikawa, S.; Hirabayashi, Y.
Glucosylceramide synthase and glycosphingolipid synthesis
Trends Cell Biol.
8
198-202
1998
Caenorhabditis elegans, Mus musculus, Rattus norvegicus, Homo sapiens (Q16739)
Itoh, M.; Kitano, T.; Watanabe, M.; Kondo, T.; Yabu, T.; Taguchi, Y.; Iwai, K.; Tashima, M.; Uchiyama, T.; Okazaki, T.
Possible role of ceramide as an indicator of chemoresistance: decrease of the ceramide content via activation of glucosylceramide synthase and sphingomyelin synthase in chemoresistant leukemia
Clin. Cancer Res.
9
415-423
2003
Homo sapiens
Marks, D.L.; Wu, K.; Paul, P.; Kamisaka, Y.; Watanabe, R.; Pagano, R.E.
Oligomerization and topology of the Golgi membrane protein glucosylceramide synthase
J. Biol. Chem.
274
451-456
1999
Homo sapiens, Rattus norvegicus
Wu, K.; Marks, D.L.; Watanabe, R.; Paul, P.; Rajan, N.; Pagano, R.E.
Histidine-193 of rat glucosylceramide synthase resides in a UDP-glucose-and inhibitor (D-threo-1-phenyl-2-decanoylamino-3-morpholinopropan-1-ol)-binding region: a biochemical and mutational study
Biochem. J.
341
395-400
1999
Homo sapiens (Q16739), Rattus norvegicus (Q9R0E0)
-
Shayman, J.A.; Abe, A.
Glucosylceramide synthase: assay and properties
Methods Enzymol.
311
42-49
2000
Canis lupus familiaris, Homo sapiens, Homo sapiens (Q16739)
Marks, D.L.; Paul, P.; Kamisaka, Y.; Pagano, R.E.
Methods for studying glucosylceramide synthase
Methods Enzymol.
311
50-59
2000
Gallus gallus, Homo sapiens, Homo sapiens (Q16739), Mus musculus, Rattus norvegicus, Rattus norvegicus (Q9R0E0), Rattus norvegicus Sprague-Dawley, Sus scrofa
Leipelt, M.; Warnecke, D.C.; Hube, B.; Zahringer, U.; Heinz, E.
Characterization of UDP-glucose: ceramide glucosyltransferases from different organisms
Biochem. Soc. Trans.
28
751-752
2000
Caenorhabditis elegans, Candida albicans, Komagataella pastoris
Marks, D.L.; Dominguez, M.; Wu, K.; Pagano, R.E.
Identification of active site residues in glucosylceramide synthase. A nucleotide-binding/catalytic motif conserved with processive beta-glycosyltransferases
J. Biol. Chem.
276
26492-26498
2001
Rattus norvegicus (Q9R0E0)
Saito, M.; Fukushima, Y.; Tatsumi, K.; Bei, L.; Fujiki, Y.; Iwamori, M.; Igarashi, T.; Sakakihara, Y.
Molecular cloning of Chinese hamster ceramide glucosyltransferase and its enhanced expression in peroxisome-defective mutant Z65 cells
Arch. Biochem. Biophys.
403
171-178
2002
Cricetulus griseus
Hayashi, Y.; Horibata, Y.; Sakaguchi, K.; Okino, N.; Ito, M.
A sensitive and reproducible assay to measure the activity of glucosylceramide synthase and lactosylceramide synthase using HPLC and fluorescent substrates
Anal. Biochem.
345
181-186
2005
Danio rerio, Cricetulus griseus
Hillig, I.; Warnecke, D.; Heinz, E.
An inhibitor of glucosylceramide synthase inhibits the human enzyme, but not enzymes from other organisms
Biosci. Biotechnol. Biochem.
69
1782-1785
2005
Homo sapiens
Di Sano, F.; Fazi, B.; Citro, G.; Lovat, P.E.; Cesareni, G.; Piacentini, M.
Glucosylceramide synthase and its functional interaction with RTN-1C regulate chemotherapeutic-induced apoptosis in neuroepithelioma cells
Cancer Res.
63
3860-3865
2003
Homo sapiens
Couto, A.S.; Caffaro, C.; Uhrig, M.L.; Kimura, E.; Peres, V.J.; Merino, E.F.; Katzin, A.M.; Nishioka, M.; Nonami, H.; Erra-Balsells, R.
Glycosphingolipids in Plasmodium falciparum. Presence of an active glucosylceramide synthase
Eur. J. Biochem.
271
2204-2214
2004
Plasmodium falciparum
Hillig, I.; Leipelt, M.; Ott, C.; Zahringer, U.; Warnecke, D.; Heinz, E.
Formation of glucosylceramide and sterol glucoside by a UDP-glucose-dependent glucosylceramide synthase from cotton expressed in Pichia pastoris
FEBS Lett.
553
365-369
2003
Gossypium arboreum
Kohyama-Koganeya, A.; Sasamura, T.; Oshima, E.; Suzuki, E.; Nishihara, S.; Ueda, R.; Hirabayashi, Y.
Drosophila glucosylceramide synthase: a negative regulator of cell death mediated by proapoptotic factors
J. Biol. Chem.
279
35995-36002
2004
Drosophila melanogaster
Gouaze, V.; Yu, J.Y.; Bleicher, R.J.; Han, T.Y.; Liu, Y.Y.; Wang, H.; Gottesman, M.M.; Bitterman, A.; Giuliano, A.E.; Cabot, M.C.
Overexpression of glucosylceramide synthase and P-glycoprotein in cancer cells selected for resistance to natural product chemotherapy
Mol. Cancer Ther.
3
633-639
2004
Homo sapiens
Aerts, J.M.; Ottenhoff, R.; Powlson, A.S.; Grefhorst, A.; van Eijk, M.; Dubbelhuis, P.F.; Aten, J.; Kuipers, F.; Serlie, M.J.; Wennekes, T.; Sethi, J.K.; ORahilly, S.; Overkleeft, H.S.
Pharmacological inhibition of glucosylceramide synthase enhances insulin sensitivity
Diabetes
56
1341-1349
2007
Mus musculus
Jennemann, R.; Sandhoff, R.; Langbein, L.; Kaden, S.; Rothermel, U.; Gallala, H.; Sandhoff, K.; Wiegandt, H.; Groene, H.J.
Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis
J. Biol. Chem.
282
3083-3094
2007
Mus musculus
Rittershaus, P.C.; Kechichian, T.B.; Allegood, J.C.; Merrill, A.H.; Hennig, M.; Luberto, C.; Del Poeta, M.
Glucosylceramide synthase is an essential regulator of pathogenicity of Cryptococcus neoformans
J. Clin. Invest.
116
1651-1659
2006
Cryptococcus neoformans (Q58FH5)
McEachern, K.A.; Fung, J.; Komarnitsky, S.; Siegel, C.S.; Chuang, W.L.; Hutto, E.; Shayman, J.A.; Grabowski, G.A.; Aerts, J.M.; Cheng, S.H.; Copeland, D.P.; Marshall, J.
A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease
Mol. Genet. Metab.
91
259-267
2007
Mus musculus
Faugeroux, V.; Genisson, Y.; Andrieu-Abadie, N.; Colie, S.; Levade, T.; Baltas, M.
C-Alkyl 5-membered ring imino sugars as new potent cytotoxic glucosylceramide synthase inhibitors
Org. Biomol. Chem.
4
4437-4439
2006
Mus musculus
Jennemann, R.; Sandhoff, R.; Wang, S.; Kiss, E.; Gretz, N.; Zuliani, C.; Martin-Villalba, A.; Jaeger, R.; Schorle, H.; Kenzelmann, M.; Bonrouhi, M.; Wiegandt, H.; Groene, H.J.
Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth
Proc. Natl. Acad. Sci. USA
102
12459-12464
2005
Mus musculus
Boucheron, C.; Desvergnes, V.; Compain, P.; Martin, O.R.; Lavi, A.; Mackeen, M.; Wormald, M.; Dwek, R.; Butters, T.D.
Design and synthesis of imino-sugar-based inhibitors of glucosylceramide synthase: the search for new therapeutic agents against Gaucher disease
Tetrahedron
16
1747-1756
2005
Homo sapiens
-
Marks, N.; Berg, M.J.; Saito, M.; Saito, M.
Glucosylceramide synthase decrease in frontal cortex of Alzheimer brain correlates with abnormal increase in endogenous ceramides: consequences to morphology and viability on enzyme suppression in cultured primary neurons
Brain Res.
1191
136-147
2008
Homo sapiens, Rattus norvegicus
Li, H.; Liu, T.; Zhang, Y.; Favre, S.; Bello, C.; Vogel, P.; Butters, T.D.; Oikonomakos, N.G.; Marrot, J.; Bleriot, Y.
New synthetic seven-membered 1-azasugars displaying potent inhibition towards glycosidases and glucosylceramide transferase
ChemBioChem
9
253-260
2008
Homo sapiens
Dbaibo, G.S.; Kfoury, Y.; Darwiche, N.; Panjarian, S.; Kozhaya, L.; Nasr, R.; Abdallah, M.; Hermine, O.; El-Sabban, M.; de The, H.; Bazarbachi, A.
Arsenic trioxide induces accumulation of cytotoxic levels of ceramide in acute promyelocytic leukemia and adult T-cell leukemia/lymphoma cells through de novo ceramide synthesis and inhibition of glucosylceramide synthase activity
Haematologica
92
753-762
2007
Homo sapiens
Xie, P.; Shen, Y.F.; Shi, Y.P.; Ge, S.M.; Gu, Z.H.; Wang, J.; Mu, H.J.; Zhang, B.; Qiao, W.Z.; Xie, K.M.
Overexpression of glucosylceramide synthase in associated with multidrug resistance of leukemia cells
Leuk. Res.
32
475-480
2008
Homo sapiens
Ramamoorthy, V.; Cahoon, E.B.; Li, J.; Thokala, M.; Minto, R.E.; Shah, D.M.
Glucosylceramide synthase is essential for alfalfa defensin-mediated growth inhibition but not for pathogenicity of Fusarium graminearum
Mol. Microbiol.
66
771-786
2007
Fusarium graminearum
Wennekes, T.; Lang, B.; Leeman, M.; van der Marel, G.A.; Smits, E.; Weber, M.; van Wiltenburg, J.; Wolberg, M.; Aerts, J.M.; Overkleeft, H.S.
Large-Scale Synthesis of the Glucosylceramide Synthase Inhibitor N-[5-(Adamantan-1-yl-methoxy)-pentyl]-1-deoxynojirimycin
Org. Process Res. Dev.
12
414-423
2008
Homo sapiens
-
Saadat, L.; Dupree, J.L.; Kilkus, J.; Han, X.; Traka, M.; Proia, R.L.; Dawson, G.; Popko, B.
Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT-deficient mice
Glia
58
391-398
2010
Mus musculus (O88693)
Niimura, Y.; Moue, T.; Takahashi, N.; Nagai, K.I.
Modification of sphingoglycolipids and sulfolipids in kidney cell lines under heat stress: Activation of monohexosylceramide synthesis as a ceramide scavenger
Glycobiology
20
710-717
2010
Chlorocebus aethiops, Canis lupus familiaris, Chlorocebus sabaeus
Crespo, P.M.; Silvestre, D.C.; Gil, G.A.; Maccioni, H.J.; Daniotti, J.L.; Caputto, B.L.
c-Fos activates glucosylceramide synthase and glycolipid synthesis in PC12 cells
J. Biol. Chem.
283
31163-31171
2008
Homo sapiens
Ruckhaeberle, E.; Karn, T.; Hanker, L.; Gaetje, R.; Metzler, D.; Holtrich, U.; Kaufmann, M.; Rody, A.
Prognostic relevance of glucosylceramide synthase (GCS) expression in breast cancer
J. Cancer Res. Clin. Oncol.
135
81-90
2009
Homo sapiens
Marza, E.; Simonsen, K.T.; Faergeman, N.J.; Lesa, G.M.
Expression of ceramide glucosyltransferases, which are essential for glycosphingolipid synthesis, is only required in a small subset of C. elegans cells
J. Cell Sci.
122
822-833
2009
Caenorhabditis elegans, Caenorhabditis elegans (O18037), Caenorhabditis elegans (Q21054)
Gupta, V.; Patwardhan, G.A.; Zhang, Q.J.; Cabot, M.C.; Jazwinski, S.M.; Liu, Y.Y.
Direct quantitative determination of ceramide glycosylation in vivo: a new approach to evaluate cellular enzyme activity of glucosylceramide synthase (GlcT-1)
J. Lipid Res.
51
866-874
2010
Homo sapiens
Hisaki, H.; Okazaki, T.; Kubota, M.; Nakane, M.; Fujimaki, T.; Nakayama, H.; Nakagomi, T.; Tamura, A.; Masuda, H.
L-PDMP improves glucosylceramide synthesis and behavior in rats with focal ischemia
Neurol. Res.
30
979-984
2008
Rattus norvegicus
Patwardhan, G.; Zhang, Q.; Yin, D.; Gupta, V.; Bao, J.; Senkal, C.; Ogretmen, B.; Cabot, M.; Shah, G.; Sylvester, P.; Jazwinski, S.; Liu, Y.
A new mixed-backbone oligonucleotide against glucosylceramide synthase sensitizes multidrug-resistant tumors to apoptosis
PLoS ONE
4
e6938
2009
Homo sapiens, Mus musculus
Wennekes, T.; van den Berg, R.; Bonger, K.; Donker-Koopman, W.; Ghisaidoobe, A.; van der Marel, G.; Strijland, A.; Aerts, J.; Overkleeft, H.
Synthesis and evaluation of dimeric lipophilic iminosugars as inhibitors of glucosylceramide metabolism
Tetrahedron Asymmetry
20
836-846
2009
Mus musculus
-
Haynes, T.A.; Filippov, V.; Filippova, M.; Yang, J.; Zhang, K.; Duerksen-Hughes, P.J.
DNA damage induces down-regulation of UDP-glucose ceramide glucosyltransferase, increases ceramide levels and triggers apoptosis in p53-deficient cancer cells
Biochim. Biophys. Acta
1821
943-953
2012
Homo sapiens
Nomura, K.H.; Murata, D.; Hayashi, Y.; Dejima, K.; Mizuguchi, S.; Kage-Nakadai, E.; Gengyo-Ando, K.; Mitani, S.; Hirabayashi, Y.; Ito, M.; Nomura, K.
Ceramide glucosyltransferase of the nematode Caenorhabditis elegans is involved in oocyte formation and in early embryonic cell division
Glycobiology
21
834-848
2011
Caenorhabditis elegans
Gupta, V.; Bhinge, K.N.; Hosain, S.B.; Xiong, K.; Gu, X.; Shi, R.; Ho, M.Y.; Khoo, K.H.; Li, S.C.; Li, Y.T.; Ambudkar, S.V.; Jazwinski, S.M.; Liu, Y.Y.
Ceramide glycosylation by glucosylceramide synthase selectively maintains the properties of breast cancer stem cells
J. Biol. Chem.
287
37195-37205
2012
Homo sapiens
Kohyama-Koganeya, A.; Nabetani, T.; Miura, M.; Hirabayashi, Y.
Glucosylceramide synthase in the fat body controls energy metabolism in Drosophila
J. Lipid Res.
52
1392-1399
2011
Drosophila melanogaster
Ghisaidoobe, A.T.; van den Berg, R.J.; Butt, S.S.; Strijland, A.; Donker-Koopman, W.E.; Scheij, S.; van den Nieuwendijk, A.M.; Koomen, G.J.; van Loevezijn, A.; Leemhuis, M.; Wennekes, T.; van der Stelt, M.; van der Marel, G.A.; van Boeckel, C.A.; Aerts, J.M.; Overkleeft, H.S.
Identification and development of biphenyl substituted iminosugars as improved dual glucosylceramide synthase/neutral glucosylceramidase inhibitors
J. Med. Chem.
57
9096-9104
2014
Homo sapiens (Q16739)
Niino, S.; Nakamura, Y.; Hirabayashi, Y.; Nagano-Ito, M.; Ichikawa, S.
A small molecule inhibitor of Bcl-2, HA14-1, also inhibits ceramide glucosyltransferase
Biochem. Biophys. Res. Commun.
433
170-174
2013
Homo sapiens (Q16739)
van den Berg, R.J.; van Rijssel, E.R.; Ferraz, M.J.; Houben, J.; Strijland, A.; Donker-Koopman, W.E.; Wennekes, T.; Bonger, K.M.; Ghisaidoobe, A.B.; Hoogendoorn, S.; van der Marel, G.A.; Codee, J.D.; Overkleeft, H.S.; Aerts, J.M.
Synthesis and evaluation of hybrid structures composed of two glucosylceramide synthase inhibitors
ChemMedChem
10
2042-2062
2015
Homo sapiens (Q16739)
Alam, S.; Fedier, A.; Kohler, R.S.; Jacob, F.
Glucosylceramide synthase inhibitors differentially affect expression of glycosphingolipids
Glycobiology
25
351-356
2015
Homo sapiens (Q16739)
Ashe, K.M.; Budman, E.; Bangari, D.S.; Siegel, C.S.; Nietupski, J.B.; Wang, B.; Desnick, R.J.; Scheule, R.K.; Leonard, J.P.; Cheng, S.H.; Marshall, J.
Efficacy of enzyme and substrate reduction therapy with a novel antagonist of glucosylceramide synthase for Fabry disease
Mol. Med.
21
389-399
2015
Mus musculus (O88693), Mus musculus 129SvEv (O88693)
Chiu, W.H.; Su, W.C.; Li, C.L.; Chen, C.L.; Lin, C.F.
An increase in glucosylceramide synthase induces Bcl-xL-mediated cell survival in vinorelbine-resistant lung adenocarcinoma cells
Oncotarget
6
20513-20524
2015
Homo sapiens (Q16739)
Wegner, M.S.; Schoemel, N.; Gruber, L.; Oertel, S.B.; Kjellberg, M.A.; Mattjus, P.; Kurz, J.; Trautmann, S.; Peng, B.; Wegner, M.; Kaulich, M.; Ahrends, R.; Geisslinger, G.; Groesch, S.
UDP-glucose ceramide glucosyltransferase activates AKT, promoted proliferation, and doxorubicin resistance in breast cancer cells
Cell. Mol. Life Sci.
75
3393-3410
2018
Homo sapiens (Q16739)
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