Sushi repeat-containing protein SRPX2 (SRPX2, SRPUL)

Function: - role in the perisylvian region, critical for language & cognitive development Structure: - contains 1 HYR domain - contains 3 Sushi (CCP/SCR) domains Compartment: secreted Pathology: - defects in SRPX2 are a cause of bilateral perisylvian polymicrogyria - defects in SRPX2 are a cause of X-linked rolandic epilepsy with speech dyspraxia & mental retardation

Properties

SIZE: MW = 53 kD entity length = 465 aa COMPARTMENT: extracellular compartment MOTIF: signal sequence {1-23} Sushi domain {69-119} MOTIF: cysteine residue {C71} MODIFICATION: cysteine residue {C105} cysteine residue {C91} MODIFICATION: cysteine residue {C117} cysteine residue {C105} MODIFICATION: cysteine residue {C71} cysteine residue {C117} MODIFICATION: cysteine residue {C91} Sushi domain {120-178} MOTIF: cysteine residue {C122} MODIFICATION: cysteine residue {C163} cysteine residue {C149} MODIFICATION: cysteine residue {C176} cysteine residue {C163} MODIFICATION: cysteine residue {C122} cysteine residue {C176} MODIFICATION: cysteine residue {C149} HYR {177-261} Sushi domain {262-321} MOTIF: cysteine residue {C264} MODIFICATION: cysteine residue {C306} cysteine residue {C292} MODIFICATION: cysteine residue {C319} cysteine residue {C306} MODIFICATION: cysteine residue {C264} cysteine residue {C319} MODIFICATION: cysteine residue {C292}

Database Correlations

OMIM 300388 UniProt O60687 PFAM correlations

References

UniProt :accession O60687