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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