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Literature summary for 2.7.1.108 extracted from

  • Kranz, C.; Jungeblut, C.; Denecke, J.; Erlekotte, A.; Sohlbach, C.; Debus, V.; Kehl, H.G.; Harms, E.; Reith, A.; Reichel, S.; Gröbe, H.; Hammersen, G.; Schwarzer, U.; Marquardt, T.
    A defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy (2007), Am. J. Hum. Genet., 80, 433-440.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 2.7.1.108

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
CTP + dolichol Homo sapiens DK1 is responsible for the final step of the de novo biosynthesis of dolichol phosphate CDP + dolichyl phosphate
-
 ?
CTP + dolichol Homo sapiens DK1 is responsible for the final step of the de novo biosynthesis of dolichol phosphate, defect in human DK1 affects the biosynthesis of dolichol phosphate by the disturbance of the final phosphorylation step, causes lethal phenotype with death in early infancy. CDP + dolichyl phosphate dolichyl phosphate is involved in several glycosylation reactions, such as N-glycosylation, glycosylphosphatidylinositol (GPI)-anchor biosynthesis, and C- and O-mannosylation ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
 extracts gained from patient fibroblasts show remarkably lower enzyme activity than extracts from control cells, in comparison with controls, the decrease in activity was 94.5-98.6% for all patients Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
CTP + dolichol DK1 is responsible for the final step of the de novo biosynthesis of dolichol phosphate Homo sapiens CDP + dolichyl phosphate
-
 ?
CTP + dolichol a defect in dolichol phosphate biosynthesis causes a new inherited disorder with death in early infancy Homo sapiens CDP + dolichyl phosphate
-
 ?
CTP + dolichol DK1 is responsible for the final step of the de novo biosynthesis of dolichol phosphate, defect in human DK1 affects the biosynthesis of dolichol phosphate by the disturbance of the final phosphorylation step, causes lethal phenotype with death in early infancy. Homo sapiens CDP + dolichyl phosphate dolichyl phosphate is involved in several glycosylation reactions, such as N-glycosylation, glycosylphosphatidylinositol (GPI)-anchor biosynthesis, and C- and O-mannosylation ?

Synonyms

Synonyms Comment Organism
DK1
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
CTP
-
 Homo sapiens