EC 1.1.1.441: polyprenol dehydrogenase [NAD(P)H]
This is an abbreviated version!
For detailed information about polyprenol dehydrogenase [NAD(P)H], go to the full flat file.
Reaction
Synonyms
chDHRSX, DHRSX, dolichal reductase
ECTree
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| Results | in table |
|---|---|
| 576 | AA Sequence |
| 2 | Cloned(Commentary) |
| 4 | Cofactor |
| 3 | General Information |
| 2 | Inhibitors |
| 12 | KM Value [mM] |
| 1 | Localization |
| 6 | Natural Substrates/ Products (Substrates) |
| 2 | Organism |
| 2 | Purification (Commentary) |
| 2 | Reaction |
| 2 | Reference |
| 2 | Source Tissue |
| 18 | Substrates and Products (Substrate) |
| 4 | Synonyms |
| 1 | Systematic Name |
| 12 | Turnover Number [1/s] |
General Information
General Information on EC 1.1.1.441 - polyprenol dehydrogenase [NAD(P)H]
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malfunction
physiological function
the enzyme catalyzes two nonconsecutive steps in dolichol synthesis pathway using different cofactor. The enzyme catalyses the oxidation of of polyprenol using NAD+ as the electron acceptor, and the reduction of dolichal using NADPH as the electron donors
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absence of the dolichol synthesis gene DHRSX leads to N-glycosylation defects in Lec5 and Lec9 Chinese hamster ovary cells
malfunction
DHRSX deficiency leads to defective N-glycosylation by disrupting dolichol synthesis. Patients with missense variants in DHRSX (DHRSX-CDG) show a pseudoautosomal-recessive disease presenting as a congenital disorder of glycosylation

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