DCPS Chromosome 11

Decapping enzyme, scavenger
10 variants 10 Health Risk

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What This Gene Does
This gene encodes a member of the histidine triad family of pyrophosphatases that removes short mRNA fragments containing the 5′ mRNA cap structure, which appear in the 3′ → 5′ mRNA decay pathway, following deadenylation and exosome-mediated turnover. This enzyme hydrolyzes the triphosphate linkage of the cap structure (7-methylguanosine nucleoside triphosphate) to yield 7-methylguanosine monophosphate and nucleoside diphosphate. It protects the cell from the potentially toxic accumulation of these short, capped mRNA fragments, and regulates the activity of other cap-binding proteins, which are inhibited by their accumulation. It also acts as a transcript-specific modulator of pre-mRNA splicing and microRNA turnover. [provided by RefSeq, Apr 2017]
Gene Info
Gene Group
Histidine triad superfamily
Locus Type
gene with protein product
Location
11q24.2
Ensembl
ENSG00000110063
Associated Conditions (2)
Al-Raqad syndrome
Inborn genetic diseases
Key Variants
All Variants (10)
RSID Category Clinical Significance Conditions
RS137941190 Health Risk Conflicting classifications of pathogenicity Al-Raqad syndrome, Al-Raqad syndrome
RS140377449 Health Risk Conflicting classifications of pathogenicity
RS147935593 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Al-Raqad syndrome, Inborn genetic diseases
RS2497258001 Health Risk Likely pathogenic Al-Raqad syndrome, Al-Raqad syndrome
RS556401323 Health Risk Likely pathogenic
RS768872827 Health Risk Likely pathogenic Al-Raqad syndrome, Al-Raqad syndrome
RS904572688 Health Risk Likely pathogenic Al-Raqad syndrome, Al-Raqad syndrome
RS1057519083 Health Risk Pathogenic Al-Raqad syndrome, Al-Raqad syndrome
RS138737928 Health Risk Pathogenic Al-Raqad syndrome, Al-Raqad syndrome
RS770528538 Health Risk Pathogenic Al-Raqad syndrome, Al-Raqad syndrome
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