Ask about this productRelated genes to: NEU2 antibody
- Gene:
- NEU2 NIH gene
- Name:
- neuraminidase 2
- Previous symbol:
- -
- Synonyms:
- SIAL2
- Chromosome:
- 2q37.1
- Locus Type:
- gene with protein product
- Date approved:
- 1998-02-26
- Date modifiied:
- 2016-10-05
Related products to: NEU2 antibody
Related articles to: NEU2 antibody
- This chapter offers an overview of the development of BTP3-Neu5Ac and the functions of sialidase, as revealed through its use. Imaging of sialidase activity in the brain with BTP3-Neu5Ac has demonstrated that high sialidase activity is primarily detected in the white matter. In the hippocampus, a region critical for memory, glutamatergic nerve terminals exhibit particularly high sialidase activity. Based on the sialidase activity atlas, it was found that sialidase plays a crucial role in cognitive function within the hippocampus. Real-time imaging of changes in brain sialidase activity using BTP3-Neu5Ac has shown that sialidase activity at nerve terminals can rapidly increase within seconds in response to neural activity. This rapid fluctuation in sialidase activity contributes to a negative feedback mechanism on excitatory neurotransmission by sialidase. Additionally, studies on the distribution of sialidase activity in the pancreas have shown that the sialidase inhibitor 2,3-dehydro-2-deoxy-N-acetylneuraminic acid (DANA) promotes insulin secretion. Importantly, DANA does not induce insulin secretion during hypoglycemia, suggesting its potential for diabetes treatment with a lower risk of hypoglycemic side effects. Moreover, research into the distribution of sialidase activity in the skin has revealed that the sialidase isozyme NEU2 promotes elastin production. Thus, information on the distribution of sialidase activity in tissues provides valuable insights into the function of sialidase and supports the development of sialidase-targeted drugs. - Source: PubMed
Hata ShotaAnuar Azliza MadKurebayashi YuukiTakahashi TadanobuTakeuchi HideyukiOtsubo TadamuneMinami Akira - Neuraminidase 1 (NEU1) is a lysosomal sialidase that removes terminal α-bound sialic acid from sialylglycoconjugates and contributes to ubiquitous catabolism of sialylglycoconjugates and immunoregulatory functions. Different from other human sialidases, including NEU2 to NEU4, NEU1 is first produced as an N-glycosylated precursor protein, which binds to its protective protein/cathepsin A (CTSA) and then forms a lysosomal multienzyme complex (LMC) with β-galactosidase 1 (GLB1) in the rough endoplasmic reticulum (RER) lumen. NEU1 trafficking to lysosomes and intralysosomal activation under acidic pH conditions essentially requires association with CTSA, which carries terminal mannose 6-phosphate (M6P)-type N-glycan to bind with cation-dependent (CD) M6P receptor (CD-M6PR) in the Golgi apparatus via endosomes. In contrast, the single NEU1 gene overexpression in mammalian cells results in NEU1 protein crystallization in the RER owing to self-aggregation at a relatively low intrinsic CTSA level. Two NEU1 deficiencies, sialidosis (SiD) and galactosialidosis (GS), are caused by autosomal recessive NEU1 and CTSA gene mutations, respectively. These untreatable disorders are associated with excessive storage of sialylglycans in neurovisceral organs and systemic symptoms. We produced a new GS model mouse by introducing a homozygous Ctsa IVS6+1g/a mutation into the murine gene locus, leading to partial exon 6 skipping and simultaneous deficiency of Ctsa and Neu1. The GS mice exhibited clinical symptoms similar to those seen in juvenile/adult GS patients, including myoclonic seizures, suppressed behavior, a gargoyle-like face, edema, proctoptosis owing to Neu1 deficiency, and sialylglycan accumulation related to neurovisceral inflammation. Evaluating the efficacy of a novel therapy utilizing GS and SiD model mice and overcoming the human NEU1 gene product shortage will be necessary for a novel, effective treatment for NEU1 deficiencies. - Source: PubMed
Itoh KohjiTsukimoto Jun - Multiple sclerosis (MS) is an autoimmune demyelinating disease of the central nervous system (CNS) characterized by multifocal inflammation and axonal degeneration, driven by innate and adaptive immune cells. The Janus Kinase (JAK)/Signal Transducers and Activators of Transcription (STAT)/Suppressors Of Cytokine Signaling (SOCS) pathway regulates immune cell activity, with SOCS proteins functioning as negative regulators. Using the Experimental Autoimmune Encephalomyelitis (EAE) model of MS, our prior work demonstrated that mice lacking in myeloid cells () developed severe, brain-targeted EAE (btEAE), with increased cerebellar infiltration of activated neutrophils. To define neutrophil-specific roles, we generated mice with deletion restricted to neutrophils (). Following MOG-induced EAE, these mice exhibited clinical features identical to mice, including severe cerebellar demyelination, increased cerebellar infiltration of activated neutrophils and CD4 T-cells, and clinical symptoms of both btEAE and classical EAE (cEAE), the latter involving the spinal cord (SC). Cerebellar neutrophils from mice exhibited a primed, inflammatory phenotype with elevated reactive oxygen species, neutrophil extracellular traps (NETs) and heightened production of pro-inflammatory cytokines/chemokines. Neutrophil depletion alleviated btEAE, confirming their pathogenic role. Single-cell RNA Sequencing (scRNA-Seq) of cerebellum (CB) and SC neutrophils revealed five clusters in naïve and EAE mice, with expansion of two clusters (Neu2 and Neu4) in mice with EAE. Neu2, Neu3 and Neu4 clusters showed high expression of , and , with Neu4 enriched in cytokine signaling pathways and inflammatory responses. Strikingly, Saa3 mRNA and protein expression were markedly increased in the CB and SC of mice with EAE compared to controls. Translationally, the human orthologue SAA1 was significantly elevated in plasma from MS patients relative to healthy controls. Collectively, these findings demonstrate that deficiency unleashes pathogenic neutrophil activity in mice with EAE. They further demonstrate neutrophil heterogeneity within the inflamed CNS and define inflammatory transcriptional states, with Saa3/SAA1 as a potential biomarker and/or target in autoimmune neuroinflammation. - Source: PubMed
Publication date: 2026/03/18
Wang YongTurbitt William JZhou LiannaYan ZhaoqiPatel Sweta BYang WeiLi ZhangBuckley Jessica AMulia GraceWelner Robert SMeador William RRaman ChanderQin HongweiBenveniste Etty N - Bosch-Boonstra-Schaaf Optic Atrophy Syndrome (BBSOAS) is a rare autosomal dominant neurodevelopmental disorder caused by mutations or deletions in NR2F1, leading to intellectual disability, developmental delay, visual impairments, epilepsy, hypotonia, and autistic traits. We generated six novel human induced pluripotent stem cell (hiPSC) lines from BBSOAS patients with variable clinical phenotypes. These lines provide a versatile and renewable resource by serving as a unique platform to model NR2F1-related developmental defects in vitro and elucidate the molecular and cellular mechanisms underlying BBSOAS. Their availability will facilitate mechanistic, comparative, and therapeutic studies, advancing our understanding of NR2F1 function in human neural development. - Source: PubMed
Publication date: 2026/03/10
Bertacchi MicheleDesprat RomainLesca GaetanQuelin ChloéWillems MarjolaineVincent-Delorme CatherineFaivre LaurenceJorgensen ChristianStuder Michèle - Sepsis-induced liver injury involves profound immune dysregulation. Natural compounds such as artesunate (ART), capsaicin (CAP), and oridonin (ORI) have demonstrated efficacy in mitigating systemic inflammation; however, their comparative cellular mechanisms in sepsis remain poorly characterized. Here, we integrated and reanalyzed the single-cell transcriptomic datasets of murine livers from 5 conditions: healthy control, sepsis, and sepsis treated with ART, CAP, or ORI. We uncover a spectrum of neutrophil subtypes with treatment-responsive phenotypes, including anti-inflammatory Ngp+ Neu1, immunosuppressive Cd274+ Neu2, and mature Stfa2l1+ Neu4, in which the excessive neutrophil expansion was suppressed by all 3 therapies through distinct regulon activities. Macrophages were activated and infiltration to partially rebalance immune homeostasis. Endothelial cells underwent profound reprogramming under sepsis, marked by NF-κB activation and oxidative stress, which are selectively modulated by treatment. Cell-cell communication analysis revealed a convergent dampening of inflammatory ligand-receptor networks, including the CCL signaling axis, and therapy-specific enhancement of regenerative cues, such as EGF signaling. Our findings reveal both shared and compound-specific immunoregulatory effects of ART, CAP, and ORI, offering mechanistic insights into hepatic immune rebalancing in sepsis. This single-cell atlas provides a conceptual framework for the rational design of multitarget therapies and highlights the key immune modules amenable to therapeutic intervention. - Source: PubMed
Zhou Chen