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Domain

Composed of 3 domains: the N-terminal domain has structural similarity to S-adenosylmethionine decarboxylase, the central domain is made up of four beta strands and the C-terminal domain is similar in structure to spermidine synthase. The N- and C-terminal domains are both required for activity.

Function

Catalyzes the production of spermine from spermidine and decarboxylated S-adenosylmethionine (dcSAM).

Involvement in disease

Intellectual developmental disorder, X-linked, syndromic, Snyder-Robinson type

MRXSSR

An X-linked intellectual disability syndrome characterized by a collection of clinical features including facial asymmetry, marfanoid habitus, hypertonia, osteoporosis and unsteady gait.

None

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

Pathway

Amine and polyamine biosynthesis; spermine biosynthesis; spermine from spermidine: step 1/1.

Sequence similarities

Belongs to the spermidine/spermine synthase family.

Alternative names

Spermine synthase, SPMSY, Spermidine aminopropyltransferase, SMS

Target type

Proteins

Primary research area

Epigenetics

Molecular weight

41268Da