Rabbit CLP1 Polyclonal Antibody | anti-CLP1 antibody
CLP1 Antibody (C-term)
Weitzer, S., et al. Nature 447(7141):222-226(2007)
Paushkin, S.V., et al. Cell 117(3):311-321(2004)
Zhou, Z., et al. Nature 419(6903):182-185(2002)
de Vries, H., et al. EMBO J. 19(21):5895-5904(2000)
NCBI and Uniprot Product Information
NCBI Description
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. Mutations in this gene are associated with pontocerebellar hypoplasia type 10 (PCH10). Alternatively splice transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2014]
Uniprot Description
HEAB: 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 DNA:RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA:RNA hybrid is phosphorylated more efficiently than the DNA component. Appears to have roles in both tRNA splicing and mRNA 3'-end formation. Component of the tRNA splicing endonuclease complex. Phosphorylates the 5'-terminus of the tRNA 3'-exon during tRNA splicing; this phosphorylation event is a prerequisite for the subsequent ligation of the two exon halves and the production of a mature tRNA. Component of the pre-mRNA cleavage complex II (CF-II), which seems to be required for mRNA 3'-end formation. Also phosphorylates the 5'-terminus of exogenously introduced short interfering RNAs (siRNAs), which is a necessary prerequisite for their incorporation into the RNA- induced silencing complex (RISC). However, endogenous siRNAs and microRNAs (miRNAs) that are produced by the cleavage of dsRNA precursors by DICER1 already contain a 5'-phosphate group, so this protein may be dispensible for normal RNA-mediated gene silencing. Belongs to the Clp1 family. Clp1 subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: RNA-binding; RNA splicing; EC 2.7.1.78
Chromosomal Location of Human Ortholog: 11q12
Cellular Component: nucleoplasm; cytoplasm; nucleus; tRNA-intron endonuclease complex; mRNA cleavage factor complex
Molecular Function: ATP-dependent polydeoxyribonucleotide 5'-hydroxyl-kinase activity; polydeoxyribonucleotide kinase activity; ATP-dependent polyribonucleotide 5'-hydroxyl-kinase activity; ATP binding
Biological Process: nuclear mRNA splicing, via spliceosome; transcription from RNA polymerase II promoter; cerebellar cortex development; tRNA splicing; RNA interference, siRNA loading onto RISC; RNA splicing; RNA interference, targeting of mRNA for destruction; mRNA polyadenylation; gene expression; mRNA 3'-end processing; phosphorylation; termination of RNA polymerase II transcription; mRNA cleavage
Disease: Pontocerebellar Hypoplasia, Type 10