7BX7

Cryo-EM structure of amyloid fibril formed by hnRNPA1 low complexity domain


Protein Family Annotation Pfam Database Homepage

ChainsAccessionNameDescriptionCommentsSource
A, B, C, D, E
PF11627Heterogeneous nuclear ribonucleoprotein A1, LC domain (HnRNPA1_LC)Heterogeneous nuclear ribonucleoprotein A1, LC domainThis entry includes hnRNPA1, a nuclear factor that binds to Pol II transcripts. The family of hnRNP proteins are involved in numerous RNA-related activities [1]. hnRNPA1 is involved in the packaging of pre-mRNA into hnRNP particles, transport of poly ...This entry includes hnRNPA1, a nuclear factor that binds to Pol II transcripts. The family of hnRNP proteins are involved in numerous RNA-related activities [1]. hnRNPA1 is involved in the packaging of pre-mRNA into hnRNP particles, transport of poly(A) mRNA from the nucleus to the cytoplasm and modulation of splice site selection [2]. hnRNPA1 plays a role in the splicing of pyruvate kinase PKM by binding repressively to sequences flanking PKM exon 9, inhibiting exon 9 inclusion and resulting in exon 10 inclusion and production of the PKM M2 isoform [3]. This protein binds to the IRES and inhibits the translation of the apoptosis protease activating factor APAF1. During Enterovirus 71 (EV71) infection, APAF1 expression is essential for virus-induced apoptosis and viral particle release [4]. HnRNPA1 has two RNA recognition motifs (RRM1 and RRM2) followed by an unstructured low complexity (LC) C-terminal domain, represented in this entry [5]. This domain can mediate protein-protein and protein-RNA interactions and it is involved in amyloid aggregation, leading to neurodegenerative diseases including amyotrophic lateral sclerosis (ALS) and multisystem proteinopathy (MSP) [5].
Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

ChainsPolymerMolecular FunctionBiological ProcessCellular Component
A, B, C, D, E
Heterogeneous nuclear ribonucleoprotein A1

InterPro: Protein Family Classification InterPro Database Homepage

Pharos: Disease Associations Pharos Homepage Annotation

ChainsDrug Target  Associated Disease
A, B, C, D, E
PharosP09651