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Target intelligence / Profile preview
LAS1 like ribosome biogenesis factor (LAS1L)
- Target
- LAS1L
- Molecular classification
- Enzyme (endoribonuclease activity, specifically involved in rRNA processing via its HEPN domain), Nucleolar protein, Component of multiprotein complexes (Five Friends of Methylated CHTOP (5FMC), MLL1 complex)
01
Overview
LAS1 like ribosome biogenesis factor (LAS1L) is an evolutionarily conserved nucleolar endoribonuclease essential for the maturation of the 60S ribosomal subunit and the 28S rRNA via cleavage of the ITS2 region of precursor rRNA. It functions within nucleolar complexes (including PELP1, TEX10, NOL9, WDR18, SENP3), with dynamic regulation by SUMOylation influencing its cellular localization and activity. LAS1L is indispensable for cell proliferation, and its dysfunction leads to severe cellular consequences (cell cycle arrest, apoptosis), and is implicated in tumorigenesis as well as rare neurodevelopmental syndromes and disorders[1][2][3][7][10].
- Other names
- LAS1LLas1-likeLAS1-like ribosome biogenesis factorLas1dJ475B7.2WTSMRXSWTSFLJ12525
02
Mechanism of action
Inhibition or loss of LAS1L function disrupts rRNA processing, leads to accumulation of rRNA intermediates, increases p53 activity, and causes cell cycle arrest. Possible mechanisms in cancer relate to altered expression promoting cell proliferation and invasion[1][3][7].
03
Biological functions
rRNA processing (cleavage and maturation of ITS2 region)Ribosome biogenesis (essential for synthesis of 60S ribosomal subunit and mature 28S rRNA)Cell cycle control (depletion causes p53-dependent G1 arrest)Cell proliferation and viabilityRNA binding
04
Disease associations
Cancer (elevated expression found in certain types, including castration-resistant prostate cancer and triple-negative breast cancer, promotes tumor growth)Neurodevelopmental disorders (mutations linked to intellectual disability, autism spectrum traits, motor neuron disease)Wilson-Turner Syndrome (implicated via various clinical genetics repositories)
05
Safety considerations
Targeting ribosome biogenesis proteins like LAS1L may cause cytotoxicity via broad effects on cell proliferation and protein synthesis.Knockdown or loss of function results in cell cycle arrest and can disrupt neuronal development[1][3].
06
Biomarkers
No specific clinical biomarkers are named, but LAS1L expression or mutations may serve as research biomarkers in cancer or neurodevelopmental disorders[3][9].
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