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NUDT2

Function

Catalyzes the asymmetric hydrolysis of diadenosine 5',5'''-P1,P4-tetraphosphate (Ap4A) to yield AMP and ATP (By similarity). Exhibits decapping activity towards FAD-capped RNAs and dpCoA-capped RNAs in vitro (By similarity).

Involvement in disease

Intellectual developmental disorder with or without peripheral neuropathy

IDDPN

An autosomal recessive disorder characterized by global developmental delay appearing in infancy or early childhood, intellectual disability, and progressive sensorimotor neuropathy with associated distal weakness. Affected individuals have hypotonia and delayed walking with an unsteady gait and frequent falls. Additional features may include dysarthria and subtle facial dysmorpism.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the Nudix hydrolase family.

Alternative names

APAH1, NUDT2, Bis(5'-nucleosyl)-tetraphosphatase [asymmetrical], Nucleoside diphosphate-linked moiety X motif 2, Ap4A hydrolase, Ap4Aase, Diadenosine tetraphosphatase, Nudix motif 2

swissprot:P50583 omim:602852 entrezGene:318