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N-acetyl-D-glucosaminyl-diphosphodolichol + UDP-glucuronic acid
D-glucuronyl-1,4-N-acetyl-D-glucosaminyl-diphosphodolichol + UDP
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N,N'-diacetylchitobiosyl-diphosphodolichol
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
additional information
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N-acetyl-D-glucosaminyl-diphosphodolichol + UDP-glucuronic acid
D-glucuronyl-1,4-N-acetyl-D-glucosaminyl-diphosphodolichol + UDP
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-
?
N-acetyl-D-glucosaminyl-diphosphodolichol + UDP-glucuronic acid
D-glucuronyl-1,4-N-acetyl-D-glucosaminyl-diphosphodolichol + UDP
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-
-
-
?
N-acetyl-D-glucosaminyl-diphosphodolichol + UDP-glucuronic acid
D-glucuronyl-1,4-N-acetyl-D-glucosaminyl-diphosphodolichol + UDP
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-
-
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UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N,N'-diacetylchitobiosyl-diphosphodolichol
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-
-
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?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N,N'-diacetylchitobiosyl-diphosphodolichol
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-
-
-
?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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-
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?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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-
-
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?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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-
-
?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
second step in the biosynthesis of the unique lipid-linked core oligosaccharide
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?
UDP-N-acetyl-alpha-D-glucosamine + N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
UDP + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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additional information
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5-fluoro N-acetylglucosamine is completely inactive as a glycosyl donor
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additional information
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a short C-terminal alpha helix of Alg13 is required for interaction with Alg14 and for enzyme activity. The C-terminal three amino acids of Alg14 are required for maintaining the integrity of Alg13/Alg14 complex. They are involved in the stabilization of the interaction between the C-termini of Alg13 and Alg14. Deletion of the N-terminal alpha strand of Alg13 causes the destruction of protein, indicating the structural importance of this region in protein stability
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additional information
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a short C-terminal alpha helix of Alg13 is required for interaction with Alg14 and for enzyme activity. The C-terminal three amino acids of Alg14 are required for maintaining the integrity of Alg13/Alg14 complex. They are involved in the stabilization of the interaction between the C-termini of Alg13 and Alg14. Deletion of the N-terminal alpha strand of Alg13 causes the destruction of protein, indicating the structural importance of this region in protein stability
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Adenocarcinoma
Distinct expression and prognostic value of OTU domain-containing proteins in non-small-cell lung cancer.
Brain Diseases
ALG13 X-linked intellectual disability: New variants, glycosylation analysis, and expanded phenotypes.
Brain Diseases
ALG13-CDG with Infantile Spasms in a Male Patient Due to a De Novo ALG13 Gene Mutation.
Brain Diseases
De novo mutations in epileptic encephalopathies.
Brain Diseases
Exome sequence identified a c.320A > G ALG13 variant in a female with infantile epileptic encephalopathy with normal glycosylation and random X inactivation: Review of the literature.
Brain Diseases
High prevalence of genetic alterations in early-onset epileptic encephalopathies associated with infantile movement disorders.
Brain Diseases
Predominant and novel de novo variants in 29 individuals with ALG13 deficiency: Clinical description, biomarker status, biochemical analysis, and treatment suggestions.
Brain Diseases
The First Metabolome Analysis in Children with Epilepsy and ALG13-CDG Resulting from c.320A>G Variant.
Carcinoma
Distinct expression and prognostic value of OTU domain-containing proteins in non-small-cell lung cancer.
Carcinoma, Squamous Cell
Distinct expression and prognostic value of OTU domain-containing proteins in non-small-cell lung cancer.
Congenital Disorders of Glycosylation
ALG13 participates in epileptogenesis via regulation of GABAA receptors in mouse models.
Congenital Disorders of Glycosylation
Early and lethal neurodegeneration with myasthenic and myopathic features: A new ALG14-CDG.
Congenital Disorders of Glycosylation
X chromosome exome sequencing reveals a novel ALG13 mutation in a nonsyndromic intellectual disability family with multiple affected male siblings.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
ALG13 participates in epileptogenesis via regulation of GABAA receptors in mouse models.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
ALG13 X-linked intellectual disability: New variants, glycosylation analysis, and expanded phenotypes.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Early and lethal neurodegeneration with myasthenic and myopathic features: A new ALG14-CDG.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Girls with Seizures Due to the c.320A>G Variant in ALG13 Do Not Show Abnormal Glycosylation Pattern on Standard Testing.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Predominant and novel de novo variants in 29 individuals with ALG13 deficiency: Clinical description, biomarker status, biochemical analysis, and treatment suggestions.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
X chromosome exome sequencing reveals a novel ALG13 mutation in a nonsyndromic intellectual disability family with multiple affected male siblings.
Epilepsy
A novel ALG14 missense variant in an alive child with myopathy, epilepsy, and progressive cerebral atrophy.
Epilepsy
ALG13 Deficiency Associated with Increased Seizure Susceptibility and Severity.
Epilepsy
ALG13 participates in epileptogenesis via regulation of GABAA receptors in mouse models.
Epilepsy
Congenital Disorders of Glycosylation from a Neurological Perspective.
Epilepsy
Genomic screening in rare disorders: New mutations and phenotypes, highlighting ALG14 as a novel cause of severe intellectual disability.
Genetic Diseases, Inborn
ALG13 participates in epileptogenesis via regulation of GABAA receptors in mouse models.
Intellectual Disability
ALG13 X-linked intellectual disability: New variants, glycosylation analysis, and expanded phenotypes.
Intellectual Disability
Genomic screening in rare disorders: New mutations and phenotypes, highlighting ALG14 as a novel cause of severe intellectual disability.
Intellectual Disability
X chromosome exome sequencing reveals a novel ALG13 mutation in a nonsyndromic intellectual disability family with multiple affected male siblings.
Muscular Diseases
A novel ALG14 missense variant in an alive child with myopathy, epilepsy, and progressive cerebral atrophy.
Muscular Diseases
Trouble at the junction: When myopathy and myasthenia overlap.
Myasthenic Syndromes, Congenital
Congenital myasthenic syndromes due to mutations in ALG2 and ALG14.
Myasthenic Syndromes, Congenital
Trouble at the junction: When myopathy and myasthenia overlap.
n-acetylglucosaminyldiphosphodolichol n-acetylglucosaminyltransferase deficiency
ALG13 Deficiency Associated with Increased Seizure Susceptibility and Severity.
n-acetylglucosaminyldiphosphodolichol n-acetylglucosaminyltransferase deficiency
Predominant and novel de novo variants in 29 individuals with ALG13 deficiency: Clinical description, biomarker status, biochemical analysis, and treatment suggestions.
Neoplasms
Genomic Fabric Remodeling in Metastatic Clear Cell Renal Cell Carcinoma (ccRCC): A New Paradigm and Proposal for a Personalized Gene Therapy Approach.
Neoplasms
Phenotypic and genotypic features of a large kindred with a germline AIP variant.
Seizures
ALG13 Deficiency Associated with Increased Seizure Susceptibility and Severity.
Seizures
ALG13-CDG in a male with seizures, normal cognitive development, and normal transferrin isoelectric focusing.
Seizures
Girls with Seizures Due to the c.320A>G Variant in ALG13 Do Not Show Abnormal Glycosylation Pattern on Standard Testing.
Spasms, Infantile
ALG13 X-linked intellectual disability: New variants, glycosylation analysis, and expanded phenotypes.
Spasms, Infantile
ALG13-CDG with Infantile Spasms in a Male Patient Due to a De Novo ALG13 Gene Mutation.
Spasms, Infantile
High prevalence of genetic alterations in early-onset epileptic encephalopathies associated with infantile movement disorders.
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Turco, S.J.; Heath, E.C.
Glucuronosyl-N-acetylglucosaminyl pyrophosphoryldolichol. Formation in SV40-transformed human lung fibroblasts and biosynthesis in rat lung microsomal preparations
J. Biol. Chem.
252
2918-2928
1977
Homo sapiens, Rattus norvegicus
brenda
Kaushal, G.P.; Elbein, A.D.
Purification and properties of UDP-GlcNAc:dolichyl-pyrophosphoryl-GlcNAc transferase from mung bean seedling
Plant Physiol.
81
1086-1091
1986
Vigna radiata
brenda
McLachlan, K.R.; Krag, S.S.
Three enzymes involved in oligosaccharide-lipid assembly in chinese hamster ovary cells differ in lipid substrate preference
J. Lipid Res.
35
1861-1868
1994
Cricetulus griseus
brenda
Kean, E.L.; Niu, N.
Kinetics of formation of GlcNAc-GlcNAc-P-P-dolichol by microsomes from the retina of the embryonic chick
Glycoconjugate J.
15
11-17
1998
Gallus gallus
brenda
Kean, E.L.; Wei, Z.; Anderson, V.E.; Zhang, N.; Sayre, L.M.
Regulation of the biosynthesis of N-acetylglucosaminylpyrophosphoryldolichol, feedback and product inhibition
J. Biol. Chem.
274
34072-34082
1999
Gallus gallus
brenda
Tai, V.W.F.; O'Reilly, M.K.; Imperiali, B.
Substrate specificity of N-acetylglucosaminyl(diphosphodolichol) N-acetylglucosaminyl transferase, a key enzyme in the dolichol pathway
Bioorg. Med. Chem.
9
1133-1140
2001
Sus scrofa
brenda
Bickel, T.; Lehle, L.; Schwarz, M.; Aebi, M.; Jakob, C.A.
Biosynthesis of Lipid-linked Oligosaccharides in Saccharomyces cerevisiae: Alg13p and Alg14p form a complex required for the formation of GlcNAc2-PP-dolichol
J. Biol. Chem.
280
34500-34506
2005
Saccharomyces cerevisiae (P38242), Saccharomyces cerevisiae (P53178), Saccharomyces cerevisiae
brenda
Gao, X.; Tachikawa, H.; Sato, T.; Jigami, Y.; Dean, N.
Alg14 recruits Alg13 to the cytoplasmic face of the endoplasmic reticulum to form a novel bipartite UDP-N-acetylglucosamine transferase required for the second step of N-linked glycosylation
J. Biol. Chem.
280
36254-36262
2005
Saccharomyces cerevisiae
brenda
Hartman, M.C.; Jiang, S.; Rush, J.S.; Waechter, C.J.; Coward, J.K.
Glycosyltransferase mechanisms: impact of a 5-fluoro substituent in acceptor and donor substrates on catalysis
Biochemistry
46
11630-11638
2007
Cricetulus griseus
brenda
Gao, X.D.; Moriyama, S.; Miura, N.; Dean, N.; Nishimura, S.I.
Interaction between the C-termini of Alg13 and Alg14 mediate formation of the active UDP-N-acetylglucosamine transferase complex
J. Biol. Chem.
283
32534-32541
2008
Saccharomyces cerevisiae (P53178), Saccharomyces cerevisiae
brenda
Averbeck, N.; Gao, X.D.; Nishimura, S.; Dean, N.
Alg13p, the catalytic subunit of the endoplasmic reticulum UDP-GlcNAc glycosyltransferase, is a target for proteasomal degradation
Mol. Biol. Cell
19
2169-2178
2008
Saccharomyces cerevisiae
brenda
Wang, X.; Weldeghiorghis, T.; Zhang, G.; Imperiali, B.; Prestegard, J.H.
Solution structure of Alg13: the sugar donor subunit of a yeast N-acetylglucosamine transferase
Structure
16
965-975
2008
Saccharomyces cerevisiae (P53178), Saccharomyces cerevisiae
brenda
Noffz, C.; Keppler-Ross, S.; Dean, N.
Hetero-oligomeric interactions between early glycosyltransferases of the dolichol cycle
Glycobiology
19
472-478
2009
Mesocricetus auratus
brenda
Lu, J.; Takahashi, T.; Ohoka, A.; Nakajima, K.; Hashimoto, R.; Miura, N.; Tachikawa, H.; Gao, X.D.
Alg14 organizes the formation of a multiglycosyltransferase complex involved in initiation of lipid-linked oligosaccharide biosynthesis
Glycobiology
22
504-516
2012
Saccharomyces cerevisiae
brenda