Target intelligence / Profile preview

Dolichyldiphosphatase 1 (DOLPP1)

Target
DOLPP1
Molecular classification
Enzyme (EC 3.6.1.43), Hydrolase (phosphatase specific for pyrophosphates)
01

Overview

Dolichyldiphosphatase 1 is an enzyme located in the endoplasmic reticulum that hydrolyzes dolichyl pyrophosphate (Dol-P-P) to dolichyl phosphate (Dol-P), furnishing a key step for the recycling and maintenance of dolichol lipids required for proper N-glycosylation in eukaryotic cells. This enzymatic activity is necessary to maintain optimal levels of dolichol-linked oligosaccharides, thus enabling efficient glycoprotein biosynthesis. Mutations in DOLPP1 are implicated in congenital disorders of glycosylation, underscoring its essential role in protein processing and cell function.

Other names
DOLPP1Dolichyl pyrophosphate phosphatase 1DOPP1
02

Mechanism of action

Not applicable; the enzyme is mainly a biosynthetic regulator of dolichol metabolism and N-glycosylation, with no established drug inhibitors/modulators

03

Biological functions

N-glycosylation (biosynthesis and maturation of glycoproteins)Lipid biosynthetic processCatalytic hydrolysis of dolichyl pyrophosphate
04

Disease associations

Congenital disorder of glycosylation, type ImChronic granulomatous disease (autosomal recessive)
05

Safety considerations

Deficiency or mutation leads to impaired N-glycosylation, resulting in multisystemic disease (not a direct therapeutic target; safety issues generally relate to genetic deficiency)
06

Biomarkers

Mutational analysis for congenital disorders of glycosylation involving DOLPP1

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