Glycogenin-1

Details

Name
Glycogenin-1
Synonyms
  • 2.4.1.186
  • GN-1
  • GYG
Gene Name
GYG1
Organism
Humans
Amino acid sequence
>lcl|BSEQ0009419|Glycogenin-1
MTDQAFVTLTTNDAYAKGALVLGSSLKQHRTTRRLVVLATPQVSDSMRKVLETVFDEVIM
VDVLDSGDSAHLTLMKRPELGVTLTKLHCWSLTQYSKCVFMDADTLVLANIDDLFDREEL
SAAPDPGWPDCFNSGVFVYQPSVETYNQLLHLASEQGSFDGGDQGILNTFFSSWATTDIR
KHLPFIYNLSSISIYSYLPAFKVFGASAKVVHFLGRVKPWNYTYDPKTKSVKSEAHDPNM
THPEFLILWWNIFTTNVLPLLQQFGLVKDTCSYVNVLSDLVYTLAFSCGFCRKEDVSGAI
SHLSLGEIPAMAQPFVSSEERKERWEQGQADYMGADSFDNIKRKLDTYLQ
Number of residues
350
Molecular Weight
39383.425
Theoretical pI
Not Available
GO Classification
Functions
glycogenin glucosyltransferase activity / metal ion binding
Processes
carbohydrate metabolic process / glucose metabolic process / glycogen biosynthetic process / glycogen catabolic process / small molecule metabolic process
Components
cytosol / extracellular exosome / lysosomal lumen / membrane
General Function
Metal ion binding
Specific Function
Self-glucosylates, via an inter-subunit mechanism, to form an oligosaccharide primer that serves as substrate for glycogen synthase.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Not Available
Gene sequence
>lcl|BSEQ0013146|Glycogenin-1 (GYG1)
ATGACAGATCAGGCCTTTGTGACACTAACCACAAACGATGCCTACGCCAAAGGTGCCCTG
GTCCTGGGATCATCTCTGAAACAGCACAGGACCACCAGGAGGCTGGTCGTGCTCGCCACC
CCTCAGGTCTCAGACTCCATGAGAAAAGTTTTAGAGACAGTCTTTGATGAAGTCATCATG
GTAGATGTCTTGGACAGTGGCGATTCTGCTCATCTAACCTTAATGAAGAGGCCAGAGTTG
GGTGTCACGCTGACAAAGCTCCACTGCTGGTCGCTTACACAGTATTCAAAATGTGTATTC
ATGGATGCAGATACTCTGGTCCTAGCAAATATTGATGATCTTTTTGACAGAGAAGAATTG
TCAGCAGCACCAGACCCAGGGTGGCCTGACTGCTTCAATTCCGGAGTCTTCGTTTATCAG
CCTTCAGTTGAAACATACAATCAGCTGTTGCATCTTGCTTCTGAGCAAGGTAGTTTTGAT
GGTGGGGACCAAGGCATACTGAACACATTTTTTAGCAGCTGGGCAACAACAGATATCAGA
AAACACCTGCCGTTTATTTATAACCTAAGCAGCATCTCTATATACTCCTACCTCCCGGCA
TTTAAAGTGTTTGGTGCAAGTGCCAAAGTTGTGCATTTCCTGGGACGAGTCAAACCATGG
AATTATACTTATGATCCCAAAACAAAAAGTGTCAAAAGTGAGGCCCATGATCCCAACATG
ACTCATCCAGAGTTTCTCATCCTGTGGTGGAACATCTTTACCACCAACGTTTTACCTCTG
CTTCAACAATTTGGCCTTGTCAAAGACACCTGCTCATATGTAAATGTGGAAGATGTCTCA
GGAGCCATATCACATCTGTCCCTTGGGGAGATCCCAGCTATGGCACAGCCGTTTGTATCC
TCGGAAGAACGGAAGGAACGATGGGAACAGGGCCAGGCTGATTATATGGGAGCAGATTCC
TTTGACAACATCAAGAGGAAACTTGACACTTACCTCCAGTAG
Chromosome Location
3
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDP46976
UniProtKB Entry NameGLYG_HUMAN
HGNC IDHGNC:4699
General References
  1. Barbetti F, Rocchi M, Bossolasco M, Cordera R, Sbraccia P, Finelli P, Consalez GG: The human skeletal muscle glycogenin gene: cDNA, tissue expression and chromosomal localization. Biochem Biophys Res Commun. 1996 Mar 7;220(1):72-7. [Article]
  2. Lomako J, Mazuruk K, Lomako WM, Alonso MD, Whelan WJ, Rodriguez IR: The human intron-containing gene for glycogenin maps to chromosome 3, band q24. Genomics. 1996 May 1;33(3):519-22. [Article]
  3. van Maanen MH, Fournier PA, Palmer TN, Abraham LJ: Characterization of the human glycogenin-1 gene: identification of a muscle-specific regulatory domain. Gene. 1999 Jul 8;234(2):217-26. [Article]
  4. Zhai L, Mu J, Zong H, DePaoli-Roach AA, Roach PJ: Structure and chromosomal localization of the human glycogenin-2 gene GYG2. Gene. 2000 Jan 25;242(1-2):229-35. [Article]
  5. Muzny DM, Scherer SE, Kaul R, Wang J, Yu J, Sudbrak R, Buhay CJ, Chen R, Cree A, Ding Y, Dugan-Rocha S, Gill R, Gunaratne P, Harris RA, Hawes AC, Hernandez J, Hodgson AV, Hume J, Jackson A, Khan ZM, Kovar-Smith C, Lewis LR, Lozado RJ, Metzker ML, Milosavljevic A, Miner GR, Morgan MB, Nazareth LV, Scott G, Sodergren E, Song XZ, Steffen D, Wei S, Wheeler DA, Wright MW, Worley KC, Yuan Y, Zhang Z, Adams CQ, Ansari-Lari MA, Ayele M, Brown MJ, Chen G, Chen Z, Clendenning J, Clerc-Blankenburg KP, Chen R, Chen Z, Davis C, Delgado O, Dinh HH, Dong W, Draper H, Ernst S, Fu G, Gonzalez-Garay ML, Garcia DK, Gillett W, Gu J, Hao B, Haugen E, Havlak P, He X, Hennig S, Hu S, Huang W, Jackson LR, Jacob LS, Kelly SH, Kube M, Levy R, Li Z, Liu B, Liu J, Liu W, Lu J, Maheshwari M, Nguyen BV, Okwuonu GO, Palmeiri A, Pasternak S, Perez LM, Phelps KA, Plopper FJ, Qiang B, Raymond C, Rodriguez R, Saenphimmachak C, Santibanez J, Shen H, Shen Y, Subramanian S, Tabor PE, Verduzco D, Waldron L, Wang J, Wang J, Wang Q, Williams GA, Wong GK, Yao Z, Zhang J, Zhang X, Zhao G, Zhou J, Zhou Y, Nelson D, Lehrach H, Reinhardt R, Naylor SL, Yang H, Olson M, Weinstock G, Gibbs RA: The DNA sequence, annotation and analysis of human chromosome 3. Nature. 2006 Apr 27;440(7088):1194-8. [Article]
  6. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  7. Skurat AV, Dietrich AD, Roach PJ: Interaction between glycogenin and glycogen synthase. Arch Biochem Biophys. 2006 Dec 1;456(1):93-7. Epub 2006 Oct 10. [Article]
  8. Gauci S, Helbig AO, Slijper M, Krijgsveld J, Heck AJ, Mohammed S: Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach. Anal Chem. 2009 Jun 1;81(11):4493-501. doi: 10.1021/ac9004309. [Article]
  9. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. [Article]
  10. Bienvenut WV, Sumpton D, Martinez A, Lilla S, Espagne C, Meinnel T, Giglione C: Comparative large scale characterization of plant versus mammal proteins reveals similar and idiosyncratic N-alpha-acetylation features. Mol Cell Proteomics. 2012 Jun;11(6):M111.015131. doi: 10.1074/mcp.M111.015131. Epub 2012 Jan 5. [Article]
  11. Malfatti E, Nilsson J, Hedberg-Oldfors C, Hernandez-Lain A, Michel F, Dominguez-Gonzalez C, Viennet G, Akman HO, Kornblum C, Van den Bergh P, Romero NB, Engel AG, DiMauro S, Oldfors A: A new muscle glycogen storage disease associated with glycogenin-1 deficiency. Ann Neurol. 2014 Dec;76(6):891-8. doi: 10.1002/ana.24284. Epub 2014 Oct 31. [Article]
  12. Moslemi AR, Lindberg C, Nilsson J, Tajsharghi H, Andersson B, Oldfors A: Glycogenin-1 deficiency and inactivated priming of glycogen synthesis. N Engl J Med. 2010 Apr 1;362(13):1203-10. doi: 10.1056/NEJMoa0900661. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB03435Uridine-5'-DiphosphateexperimentalunknownDetails
DB01861Uridine diphosphate glucoseexperimentalunknownDetails