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Identification
HMDB Protein ID HMDBP03064
Secondary Accession Numbers
  • 8598
Name Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
Synonyms
  1. Asparagine-linked glycosylation protein 8 homolog
  2. Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferase
  3. Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1,3-glucosyltransferase
Gene Name ALG8
Protein Type Enzyme
Biological Properties
General Function Involved in transferase activity, transferring hexosyl groups
Specific Function Adds the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Glc(1)Man(9)GlcNAc(2)-PP-Dol (By similarity).
Pathways
  • N-Glycan biosynthesis
  • protein glycosylation
Reactions
Dolichyl b-D-glucosyl phosphate + D-Glc-alpha-(1->3)-D-Man-alpha-(1->2)-D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-[D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-(D-Man-alpha-(1->2)-D-Man-alpha-(1->6))-D-Man-alpha-(1->6)]-D-Man-beta-(1->4)-D-GlcNAc-beta-(1->4)-D-GlcNAc-diphosphodolichol → Dolichol-20 details
Dolichyl D-glucosyl phosphate + → Dolichyl phosphate + details
GO Classification
Biological Process
dolichol-linked oligosaccharide biosynthetic process
post-translational protein modification
protein N-linked glycosylation via asparagine
Cellular Component
endoplasmic reticulum membrane
integral to membrane
Component
membrane
cell part
endoplasmic reticulum membrane
organelle membrane
Function
catalytic activity
transferase activity
transferase activity, transferring hexosyl groups
transferase activity, transferring glycosyl groups
Molecular Function
alpha-1,3-mannosyltransferase activity
Cellular Location
  1. Endoplasmic reticulum membrane
  2. Multi-pass membrane protein
Gene Properties
Chromosome Location 11
Locus 11q14.1
SNPs ALG8
Gene Sequence
>1581 bp
ATGGCGGCGCTCACAATTGCCACGGGTACTGGCAATTGGTTTTCGGCTTTGGCGCTCGGG
GTGACTCTTCTCAAATGCCTTCTCATCCCCACATACCATTCCACAGATTTTGAAGTACAC
CGAAACTGGCTTGCTATCACTCACAGTTTGCCAATATCACAGTGGTATTATGAGGCAACT
TCAGAGTGGACGTTGGATTACCCCCCTTTCTTTGCATGGTTTGAGTATATCCTGTCACAT
GTTGCCAAATATTTTGATCAAGAAATGCTGAATGTCCATAATTTGAATTACTCCAGCTCA
AGGACCTTACTTTTCCAGAGATTTTCCGTCATCTTTATGGATGTACTCTTTGTGTATGCT
GTCCGTGAGTGCTGTAAATGCATTGATGGAAAAAAAGTGGGTAAAGAACTTACAGAAAAG
CCAAAATTTATTCTGTCGGTATTACTTCTGTGGAACTTCGGGTTATTAATTGTGGACCAT
ATTCATTTTCAGTACAATGGCTTTTTATTTGGATTAATGCTACTCTCCATTGCACGATTA
TTTCAGAAAAGGCATATGGAAGGAGCATTTCTCTTTGCTGTTCTCCTACATTTCAAGCAT
ATCTACCTCTATGTAGCACCAGCTTATGGTGTATATCTGCTGCGATCCTACTGTTTCACT
GCAAATAAACCAGATGGGTCTATTCGATGGAAGAGTTTCAGCTTTGTTCGTGTTATTTCC
CTGGGACTGGTTGTTTTCTTAGTTTCTGCTCTTTCATTGGGTCCTTTCCTGGCCTTGAAT
CAGCTGCCTCAAGTCTTTTCCCGACTCTTTCCTTTCAAGAGGGGCCTCTGTCATGCATAT
TGGGCTCCAAACTTCTGGGCTTTGTACAATGCTTTGGACAAAGTGCTGTCTGTCATCGGT
TTGAAATTGAAATTTCTTGATCCCAACAATATTCCCAAGGCCTCAATGACAAGTGGTTTG
GTTCAGCAGTTCCAACACACAGTCCTTCCCTCAGTGACTCCCTTGGCAACCCTCATCTGC
ACACTGATTGCCATATTGCCCTCTATTTTCTGTCTTTGGTTTAAACCCCAAGGGCCCAGA
GGCTTTCTCCGATGTCTAACTCTTTGTGCCTTGAGCTCCTTTATGTTTGGGTGGCATGTT
CATGAAAAAGCCATACTTCTAGCAATTCTCCCAATGAGCCTTTTGTCTGTGGGAAAAGCA
GGAGACGCTTCGATTTTTCTGATTCTGACCACAACAGGACATTATTCCCTCTTTCCTCTG
CTCTTCACTGCACCAGAACTTCCCATTAAAATCTTACTCATGTTACTATTCACCATATAT
AGTATTTCGTCACTGAAGACTTTATTCAGAAAAGAAAAACCTCTTTTTAATTGGATGGAA
ACTTTCTACCTGCTTGGCCTGGGGCCTCTGGAAGTCTGCTGTGAATTTGTATTCCCTTTC
ACCTCCTGGAAGGTGAAGTACCCCTTCATCCCTTTGTTACTAACCTCAGTGTATTGTGCA
GTAGGCATCACATATGCTTGGTTCAAACTGTATGTTTCAGTATTGATTGACTCTGCTATT
GGCAAGACAAAGAAACAATGA
Protein Properties
Number of Residues 526
Molecular Weight 60086.915
Theoretical pI 9.144
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
MAALTIATGTGNWFSALALGVTLLKCLLIPTYHSTDFEVHRNWLAITHSLPISQWYYEAT
SEWTLDYPPFFAWFEYILSHVAKYFDQEMLNVHNLNYSSSRTLLFQRFSVIFMDVLFVYA
VRECCKCIDGKKVGKELTEKPKFILSVLLLWNFGLLIVDHIHFQYNGFLFGLMLLSIARL
FQKRHMEGAFLFAVLLHFKHIYLYVAPAYGVYLLRSYCFTANKPDGSIRWKSFSFVRVIS
LGLVVFLVSALSLGPFLALNQLPQVFSRLFPFKRGLCHAYWAPNFWALYNALDKVLSVIG
LKLKFLDPNNIPKASMTSGLVQQFQHTVLPSVTPLATLICTLIAILPSIFCLWFKPQGPR
GFLRCLTLCALSSFMFGWHVHEKAILLAILPMSLLSVGKAGDASIFLILTTTGHYSLFPL
LFTAPELPIKILLMLLFTIYSISSLKTLFRKEKPLFNWMETFYLLGLGPLEVCCEFVFPF
TSWKVKYPFIPLLLTSVYCAVGITYAWFKLYVSVLIDSAIGKTKKQ
GenBank ID Protein 56121818
UniProtKB/Swiss-Prot ID Q9BVK2
UniProtKB/Swiss-Prot Entry Name ALG8_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_024079.4
GeneCard ID ALG8
GenAtlas ID ALG8
HGNC ID HGNC:23161
References
General References
  1. 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. [PubMed:15489334 ]
  2. Stanchi F, Bertocco E, Toppo S, Dioguardi R, Simionati B, Cannata N, Zimbello R, Lanfranchi G, Valle G: Characterization of 16 novel human genes showing high similarity to yeast sequences. Yeast. 2001 Jan 15;18(1):69-80. [PubMed:11124703 ]
  3. Chantret I, Dancourt J, Dupre T, Delenda C, Bucher S, Vuillaumier-Barrot S, Ogier de Baulny H, Peletan C, Danos O, Seta N, Durand G, Oriol R, Codogno P, Moore SE: A deficiency in dolichyl-P-glucose:Glc1Man9GlcNAc2-PP-dolichyl alpha3-glucosyltransferase defines a new subtype of congenital disorders of glycosylation. J Biol Chem. 2003 Mar 14;278(11):9962-71. Epub 2002 Dec 11. [PubMed:12480927 ]
  4. Schollen E, Frank CG, Keldermans L, Reyntjens R, Grubenmann CE, Clayton PT, Winchester BG, Smeitink J, Wevers RA, Aebi M, Hennet T, Matthijs G: Clinical and molecular features of three patients with congenital disorders of glycosylation type Ih (CDG-Ih) (ALG8 deficiency). J Med Genet. 2004 Jul;41(7):550-6. [PubMed:15235028 ]