Mar 28, 2025 · CLP1 (Cleavage Factor Polyribonucleotide Kinase Subunit 1) is a Protein Coding gene. Diseases associated with CLP1 include Pontocerebellar Hypoplasia, Type 10 and Spinal Muscular Atrophy, Type Ii. Among its related pathways are Processing of Capped Intronless Pre-mRNA and Processing of Capped Intron-Containing Pre-mRNA.

This gene encodes a member of the Clp1 family. The encoded protein is a multifunctional kinase which is a component of the tRNA splicing Endonuclease complex and a component of the pre-mRNA cleavage complex II.

Feb 9, 2025 · This gene encodes a member of the Clp1 family. The encoded protein is a multifunctional kinase which is a component of the tRNA splicing endonuclease complex and a component of the pre-mRNA cleavage complex II. This protein is implicated in tRNA, mRNA, and siRNA maturation.

Mar 10, 2013 · Here we generated kinase-dead Clp1 (Clp1K/K ) mice that show a progressive loss of spinal motor neurons associated with axonal degeneration in the peripheral nerves and denervation of...

Apr 24, 2014 · From a cohort of more than 2,000 consanguineous families with childhood neurological disease, we identified a founder mutation in four independent pedigrees in cleavage and polyadenylation factor I subunit 1 (CLP1). CLP1 is a multifunctional kinase implicated in tRNA, mRNA, and siRNA maturation.

Dec 1, 2023 · Clp1, a conserved protein, is proposed to interlink 3′-end processing, transcriptional elongation and transcription termination. However, experimental evidence defining the precise roles played by Clp1, its interacting partners from other subunits and information about its exact mechanism of action during pre-mRNA processing is lacking.

Apr 24, 2014 · Human genome analyses now identified a CLP1 homozygous missense mutation (p.R140H) in five unrelated families, leading to a loss of CLP1 interaction with the tRNA splicing endonuclease (TSEN) complex, largely reduced pre-tRNA cleavage activity, and accumulation of linear tRNA introns.

Mar 29, 2023 · The CLP1 gene encodes a component of the tRNA endonuclease complex (TSEN), which removes introns from pre-tRNA transcripts. CLP1 is a multifunctional kinase implicated in tRNA, mRNA, and siRNA maturation (summary by Karaca et …

Polynucleotide kinase that can phosphorylate the 5'-hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded …

Apr 4, 2014 · From a cohort of over 2000 consanguineous families with childhood neurological disease, we identified a founder mutation in four independent pedigrees in cleavage and polyadenylation factor I subunit (CLP1). CLP1 is a multifunctional kinase implicated in tRNA, mRNA and siRNA maturation.