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Symbol KHSRP contributors: mct/ - updated : 02-02-2018
HGNC name KH-type splicing regulatory protein
HGNC id 6316
  • a proline/glycine rich N terminal
  • a central RNA-binding domain with four domains capable of binding single-stranded nucleic acids, the so-called K-homology domains, KH1 to KH4 (third and fourth KH domains required for mRNA binding and degradation) , and responsible for its nucleic acid-binding activity
  • KH2 and KH3 interact and form the structural core of the KHSRP protein (KH23)
  • a proline, glycine, alanine and glutamine rich C terminal domain
    interspecies homolog to murine Khsrp
    homolog to rat Marta1
  • KHSRP family
  • CATEGORY regulatory , RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
  • somatodendritic cytoplasm
  • shuttle between the nucleus and cytoplasm in response to extracellular stimuli, and its nuclear-cytoplasmic distribution varies substantially depending on cell type and conditions
  • basic FUNCTION
  • involving in neuron-specific splicing of the N1 exon of SRC
  • inducing the assembly of five other proteins, including the heterogeneous nuclear ribonucleoprotein F, onto the splicing enhancer
  • may be playing a role in nucleocytoplasmic mRNA targeting
  • having functions as a limiting factor in inflammatory gene expression
  • multifunctional RNA-binding protein that has been implicated in transcriptional regulation, neuro-specific alternative splicing and mRNA decay
  • involved in the etiology of milder forms of SMA
  • multi-domain RNA-binding protein that recruits the exosome-containing mRNA degradation complex to mRNAs coding for cellular proliferation and inflammatory response factors
  • with HNRNPA1 have antagonistic roles in the post-transcriptional regulation of MIRLET7A1, MIRLET7A2, MIRLET7A3 expression
  • function of KHSRP in innate immunity by negatively regulating IFN production
  • critical role for KHSRP in regulating pro-inflammatory mediators and have implications for a wide range of CNS inflammatory and autoimmune diseases
  • likely involved in Schwann cells (SCs) and neuronal differentiation by inducing CTNNB1 mRNA decay
  • ability of KHSRP to integrate different levels of gene expression is required for proper immune response, lipid metabolism, cell-fate decisions, tissue regeneration, and DNA damage response
  • KHSRP is an important regulator of circadian expression of lipid metabolism genes in the liver likely through controlling PER2 mRNA stability
  • controls important cellular functions as different as proliferation, differentiation, metabolism, and response to infectious agents
  • likely oncogenic role for KHSRP in lung cancer
  • is a nucleic acid binding protein, which negatively regulates the stability and/or translatability of many mRNA species encoding immune-relevant proteins
  • CELLULAR PROCESS nucleotide, RNA splicing
    text RNA processing/modification
    a component
  • spliceosomal subunit
    RNA RNA binding
    small molecule
  • interacting with MAP2
  • interacts with the Tudor domain of SMN, in a CARM1 methylation-dependent fashion
  • interacting with YWHAZ (impairment of exosome recruitment is mediated by the binding of the YWHAZ protein to KHSRP phosphorylated on Ser193)
  • KHSRP is a protein involved in AU-rich elements (AREs)-mediated translational silencing
  • subcellular localization of KHSRP is regulated by competing interactions with DDX1 or 14-3-3
  • KHSRP is an important regulator of mRNA stability and axonal length that works in direct opposition to ELAVL4 to regulate the levels of GAP43 and other AU-rich element (ARE)-containing neuronal mRNAs
  • KHSRP promotes decay of PER2 mRNA through an RNA-protein interaction and show that increased PER2 expression is responsible for the phase delay in cycling of several clock genes in the absence of KHSRP
  • PTH is a major regulator of both transcription and translation, and KHSRP binds SLC34A1 mRNA but its role in PTH regulation of SLC34A1 mRNA is not clear
  • DDX17 and KHSRP influence AGO2 stability by regulating miRNA levels in the cell and that loss of DDX17/KHSRP results in a decrease of unloaded AGO2
  • interaction between the Kelch domain of Kelch-like protein 12 (KLHL12) and the C-terminal domain of KHSRP contributed to KHSRP ubiquitination
  • KHSRP promotes the down-regulation of SPRY4 by a post-transcriptional mRNA regulation
  • cell & other
    Other regulated by phosphorylation of a serine within its N-terminal KH domain (KH1)
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --other  
    dysregulated in SMA1
    tumoral     --over  
    in human lung cancer
    the absence of KHSRP likely protects against the induction of inflammatory arthritis
    Variant & Polymorphism
    Candidate gene
    Therapy target promote tumor-relevant functions by at least partly employing the microtubule-destabilizing factor stathmin and represent a new potential target structure for hepatocellular carcinoma treatment