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heterogeneous nuclear ribonucleoprotein H; hnRNP H; contains: heterogeneous nuclear ribonucleoprotein H, N-terminally processed (HNRNPH1, HNRPH, HNRPH1)

Function: - component of the heterogeneous nuclear ribonucleoprotein (hnRNP) complexes which provide the substrate for the processing events that pre-mRNAs undergo before becoming functional, translatable mRNAs in the cytoplasm - mediates pre-mRNA alternative splicing regulation - inhibits, together with CUGBP1, insulin receptor pre-mRNA exon 11 inclusion in myoblasts - binds to the insulin receptor RNA - binds poly(rG) - part of a ternary complex containing FUBP2, PTBP1, PTBP2 & HNRNPH1 - identified in the spliceosome C complex - interacts with IGF2BP1 - interacts with CUGBP1 (RNA-dependent) - interacts with MBNL1 (RNA-independent) Structure: - each quasi-RRM repeat bound poly(rG); only the N-terminal QRRM bound poly(rC) & poly(rU) - none of the repeats bound detectable amounts of poly(rA) - contains 3 RRM domain (RNA recognition motif)s Compartment: nucleus, nucleoplasm Expression: - expressed ubiquitously Pathology: - up-regulated in myotonic dystrophy pathophysiology

General

heterogeneous nuclear ribonucleoprotein (hnRNP) phosphoprotein RNA-binding protein

Properties

SIZE: entity length = 449 aa MW = 49 kD COMPARTMENT: cell nucleus MOTIF: acetylation site SITE: N-TERMINUS EFFECTOR-BOUND: acetyl RNP motif NAME: RNP motif SITE: 11-90 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif Ser phosphorylation site {S23} Ser phosphorylation site {S63} Thr phosphorylation site {T100} Ser phosphorylation site {S104} Thr phosphorylation site {T107} RNP motif NAME: RNP motif SITE: 111-188 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif domain {234-433} MOTIF: consensus repeat {234-249} Tyr phosphorylation site {Y246} RNP motif NAME: RNP motif SITE: 289-364 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif Tyr phosphorylation site {Y306} Ser phosphorylation site {S310} consensus repeat {354-392} consensus repeat {354-372} consensus repeat {374-392} consensus repeat {418-433}

Database Correlations

OMIM 601035 UniProt P31943 PFAM correlations Entrez Gene 3187 Kegg hsa:3187

References

UniProt :accession P31943

Component-of

spliceosome C complex