Ask about this productRelated genes to: LARP7 antibody
- Gene:
- LARP7 NIH gene
- Name:
- La ribonucleoprotein domain family member 7
- Previous symbol:
- -
- Synonyms:
- HDCMA18P, PIP7S, DKFZP564K112
- Chromosome:
- 4q25
- Locus Type:
- gene with protein product
- Date approved:
- 2006-07-05
- Date modifiied:
- 2015-11-18
Related products to: LARP7 antibody
Related articles to: LARP7 antibody
- The internal stem loop (ISL) of the human U6 snRNA, which catalyzes pre-mRNA splicing, contains LARP7-dependent, snoRNA-guided 2'-O-methylations and an N-methylguanosine (mG) that is required for splicing of weak splice sites. Here, we show that installation of mG by the THUMPD2-TRMT112 methyltransferase complex is one of the last maturation events during U6 snRNP biogenesis. We dissect features of THUMPD2 required for association with U6 and present an experimentally validated model of the THUMPD2-TRMT112-U6 complex. Using in vitro methylation assays as well as a newly developed mG-sensitive deoxyribozyme to monitor U6-mG levels in cellular RNAs, we reveal that 2'-O-methylations within the U6 ISL enhance methylation of G. We show that mG and the 2'-O-methylations in U6 independently and interdependently influence alternative splicing. Furthermore, our data demonstrate that 2'-O-methylations in the ISL are required for incorporation of U6 into snRNPs whereas mG influences the progression of the U6 snRNP into larger assemblies, highlighting distinct roles of these modifications during spliceosome assembly. - Source: PubMed
Publication date: 2026/04/24
Kleiber NicolePetrosyan JonnyGreve MariaThomé Chairini CDybkov OlexandrTay Laurianne L EWelp Luisa MHackert PhilippTouboul DavidStark HolgerBohnsack Markus TUrlaub HenningHerzel LydiaGraille MarcHöbartner ClaudiaBohnsack Katherine E - TGF-β1/SMAD3 signaling drives organ fibrosis, underscoring the need to identify its endogenous regulators for precision therapies. LARP7, a core component of the 7SK snRNP complex that sequesters cyclin-dependent kinase (CDK)9, has an unexplored role in kidney fibrosis and a potential interplay with TGF-β/SMAD3 signaling. - Source: PubMed
Publication date: 2026/03/27
Zhao ZiyuZhou ZhuoyuCong AnshengSu CailingChen QiuyiHuang ZhijieLiu JiachenYang ZhichenZhu JieHu ZuoyuYuan LujuanLi JinjinZhou ZhanmeiCai YanranZhang WangHou Fan FanCao Wei - Enhanced P-TEFb activity is thought to promote cell proliferation by increasing the transcriptional output of RNA polymerase II. The 7SK snRNP complex, which contains LARP7 and HEXIM1, sequesters and inhibits most cellular P-TEFb to prevent premature transcription elongation. Paradoxically, instead of exerting overgrowth effects, biallelic inactivation of LARP7 is linked to Alazami syndrome, a human neurodevelopmental disorder characterized by growth restriction and cognitive impairment. Here, we report that conditional ablation of either Larp7 or Hexim1 in the murine brain reduces the size and impairs the function of the hippocampal dentate gyrus during the neonatal period. Functional analyses reveal that increased P-TEFb activity enhances self-renewal transcriptional programs in transit-amplifying neuronal progenitor cells to limit neurogenesis in developing dentate gyri. These results demonstrate that dysregulated subtissular stem cell dynamics can reconcile increased P-TEFb activity with reduced organ growth, and suggest a translational opportunity for repurposing P-TEFb inhibitors to treat medical conditions affecting dentate gyrus size and function. - Source: PubMed
Publication date: 2026/03/23
Fang YinQiu TongWang PingBai ShujunWang MinYang ChaoWang YanZhang PeixuanWang HeLiu ShanlingXiao XueLi Qintong - Peripheral nerve injuries (PNIs) present a persistent clinical challenge because of the intrinsically limited regenerative capacity of peripheral nerves. While dental pulp stem cells (DPSCs) exhibit significant neuroregenerative potential, their therapeutic efficacy is constrained by hostile microenvironments and inherent functional heterogeneity. Genetic modification may offer a promising strategy to enhance their therapeutic capabilities. - Source: PubMed
Publication date: 2026/03/11
Yang ZihanQu GuanlinWang XipingWang LiChen LuFu GuiqiangChen WenzeYang ZitongLi WenjingZhou YuqiongJin JiachengZhou LinxiZou Duohong - Alazami syndrome is a neurodevelopmental disorder characterized by postnatal growth retardation, moderate to severe intellectual disability, and facial dysmorphology. It is caused by biallelic variants in the transcriptional regulator La ribonucleoprotein 7 (LARP7), where frameshift variants accounted for the majority of cases. The current study presents 7 new patients, including 3 males and 4 females from 3 unrelated families. Careful and thorough clinical examination identified novel oro-dental disease abnormalities, including a prominent premaxilla and enamel defects. The detected variants (c.1113_1116del, c.997 + 2T > C and c.518T > C) were not reported in the previous studies. The substitution c.518T > C represented the second missense variant to be identified in patients with Alazami syndrome. Male patients from the three families fulfilled ≥ 2 clinical warning signs of primary immunodeficiency. Lymphocyte subset counts and immunoglobulin levels were estimated in patients from two families. The values were within reference ranges, with only minor non-significant alterations in cytotoxic T-cell counts. A functional assay of B lymphocyte response was performed in one family, demonstrating impaired Streptococcus pneumoniae IgG antibody production following Pneumovax vaccination in the male patient, while his female sibling mounted an adequate response. In conclusion, the disease has a wide range of symptoms, which vary greatly among the affected patients. Our study expanded the clinical and molecular spectrum of the disorder and highlighted immunodeficiency as an underrecognized disease feature, potentially with a male sex predilection. - Source: PubMed
Publication date: 2026/03/11
Sharaf-Eldin WessamGhorab Raghda MRafat KarimaMahmoud HebaHassib NehalAlahlafi AbdelazizMaroofian RezaGleeson Joseph GEssawi MonaZaki Maha S