Recombinant Human La-related protein 7 (LARP7)

Uniprot Id

Q4G0J3

Target Species

Human

Target Name

LARP7

Target Full Name

La-related protein 7

Target Function

RNA-binding protein that specifically binds distinct small nuclear RNA (snRNAs) and regulates their processing and function. Specifically binds the 7SK snRNA (7SK RNA) and acts as a core component of the 7SK ribonucleoprotein (RNP) complex, thereby acting as a negative regulator of transcription elongation by RNA polymerase II. The 7SK RNP complex sequesters the positive transcription elongation factor b (P-TEFb) in a large inactive 7SK RNP complex preventing RNA polymerase II phosphorylation and subsequent transcriptional elongation. The 7SK RNP complex also promotes snRNA gene transcription by RNA polymerase II via interaction with the little elongation complex (LEC). LARP7 specifically binds to the highly conserved 3'-terminal U-rich stretch of 7SK RNA; on stimulation, remains associated with 7SK RNA, whereas P-TEFb is released from the complex. LARP7 also acts as a regulator of mRNA splicing fidelity by promoting U6 snRNA processing. Specifically binds U6 snRNAs and associates with a subset of box C/D RNP complexes: promotes U6 snRNA 2'-O-methylation by facilitating U6 snRNA loading into box C/D RNP complexes. U6 snRNA 2'-O-methylation is required for mRNA splicing fidelity. Binds U6 snRNAs with a 5'-CAGGG-3' sequence motif. U6 snRNA processing is required for spermatogenesis.

Target Involvement

Alazami syndrome (ALAZS)

Target Subcellular Location

Nucleus, nucleoplasm.

Target Synonyms

DKFZp564K112; HDCMA18P; La related protein 7; La ribonucleoprotein domain family member 7; La ribonucleoprotein domain family member 7; La-related protein 7; LARP 7; larp7; LARP7_HUMAN; MGC104360; P TEFb interaction protein for 7SK stability; P-TEFb-interaction protein for 7SK stability; PIP7S

Target Background

This gene encodes a protein which is found in the 7SK snRNP (small nuclear ribonucleoprotein). This snRNP complex inhibits a cyclin-dependent kinase, positive transcription elongation factor b, which is required for paused RNA polymerase II at a promoter to begin transcription elongation. A pseudogene of this gene is located on chromosome 3. Alternative splicing results in multiple transcript variants.