SMART accession number:SM01385
Description: This family contains the breast cancer tumour suppressor BRCA2-interacting protein DSS1 and its homologue SEM1, both of which are short acidic proteins. DSS1 has been shown to be a conserved component of the Rae1 mediated mRNA export pathway in Schizosaccharomyces pombe (PMID:15990877).
Interpro abstract (IPR007834):

This family includes yeast Sem1 and its mammalian homologue, DSS1.

Sem1/DSS1 (also known as rpn15) is a component of lid subcomplex of 26S proteasome regulatory subunit [(PUBMED:23643786), (PUBMED:24412063), (PUBMED:15117943)]. Besides being a subunit of the 26S proteasome, Sem1/DSS1 associates with other protein complexes [(PUBMED:26944332)]. It is a component of the nuclear pore complex (NPC)-associated TREX-2 complex that is required for transcription-coupled mRNA export, and the COP9 signalosome, which is involved in deneddylation [(PUBMED:24896180), (PUBMED:19289793), (PUBMED:26456823)].

Loss of DSS1 in humans has been associated with split hand/split foot malformations [(PUBMED:8782053)].

GO process:mRNA export from nucleus (GO:0006406), proteasome assembly (GO:0043248)
GO component:proteasome regulatory particle, lid subcomplex (GO:0008541)
Family alignment:
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There are 1265 DSS1_SEM1 domains in 1265 proteins in SMART's nrdb database.

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