CCM2 Antibody (N-term) Blocking Peptide
- Known as:
- CCM2 Antibody (N-terminus) Blocking Peptide
- Catalog number:
- BP6875a
- Product Quantity:
- 2
- Category:
- -
- Supplier:
- Abgen
- Gene target:
- CCM2 Antibody (N-term) Blocking Peptide
Related genes to: CCM2 Antibody (N-term) Blocking Peptide
- Gene:
- CCM2 NIH gene
- Name:
- CCM2 scaffold protein
- Previous symbol:
- C7orf22
- Synonyms:
- MGC4607, OSM
- Chromosome:
- 7p13
- Locus Type:
- gene with protein product
- Date approved:
- 2003-07-14
- Date modifiied:
- 2019-04-23
Related products to: CCM2 Antibody (N-term) Blocking Peptide
Related articles to: CCM2 Antibody (N-term) Blocking Peptide
- Familial cerebral cavernous malformation (FCCM) is a hereditary vascular disorder associated with pathogenic variants of , and . Among these, -related FCCM is considered the most severe subtype and is characterized by early onset, multiple lesions, frequent brainstem involvement, and an increased risk of intracranial hemorrhage. Brainstem symptoms may be the initial manifestations. However, isolated oculomotor nerve palsy in childhood is rare. We report the case of a 17-month-old girl with normal developmental milestones who developed mild right ptosis, strabismus, and impaired adduction and upward gaze of the right eye at nine months of age. There was no family history of seizures or intracranial hemorrhage. Computed tomography of the head revealed multiple intracranial calcifications. Susceptibility-weighted angiography (SWAN) magnetic resonance imaging (MRI) revealed multiple cerebral cavernous malformations (CCMs), and follow-up MRI one year later demonstrated new lesions, including pontine involvement. Fast imaging employing steady-state acquisition (FIESTA) revealed hemorrhagic changes adjacent to the right oculomotor nerve within the interpeduncular cistern, suggesting that the partial oculomotor nerve palsy was associated with focal hemorrhagic involvement. Genetic analysis revealed the presence of a novel variant. This case highlights that -related FCCM may initially present with isolated cranial neuropathy during childhood and subsequently show rapid radiological progression. Careful longitudinal follow-up is required because of the risk of future neurological deterioration. - Source: PubMed
Publication date: 2026/06/22
Murakami YoshimiInoue TakanobuIshikura ShunFujimoto RyoAoyama Hiromi - Cerebral cavernous malformations (CCMs) are vascular anomalies prone to hemorrhage, leading to neurological deficits and reduced quality of life. Current therapies are limited, and molecular mechanisms underlying vascular instability remain incompletely understood. MicroRNAs have emerged as key regulators of vascular integrity. This study investigates the role of microRNA-21-3p (miR-21-3p) in modulating oxidative stress and angiogenesis in CCM. - Source: PubMed
Publication date: 2026/07/20
Guo Xin-XingHuang Zhong-RunChen Pei-ShengLi QiLi JiaShi Zhong-Song - Actin cytoskeleton dysregulation contributes to vascular anomalies such as cerebral cavernous malformation (CCM). Talin rod domain containing-1 (TLNRD1) has been reported to interact with cerebral cavernous malformations 2 protein (CCM2), yet the downstream signaling remains debated, as previous studies have described opposite directions of Krueppel-like factor 2/4 (KLF2/4) changes after TLNRD1 depletion. Here, we combined biochemical analyses, structural modeling, transcriptomics, and single-cell network perturbation to examine the TLNRD1-CCM2 axis in endothelial cells. Coimmunoprecipitation and mass spectrometry confirmed the association between TLNRD1 and the CCM complex. Furthermore, protein docking predicted a stable TLNRD1-CCM2 interface (ΔG ≈ -50.36 kcal/mol) supported by prominent hydrogen bonds. Bulk RNA sequencing following TLNRD1 knockdown identified 677 differentially expressed genes, which were heavily enriched for actin cytoskeleton organization, with limited support for activation of the canonical MEKK3-KLF2/4 program. To assess KLF2/4 more directly, we analyzed human CCM single-cell RNA sequencing using scTenifoldKnk alongside complementary in vitro perturbations. Across these orthogonal analyses, KLF2 and KLF4 showed little to no consistent transcriptional alterations. Instead, TLNRD1 perturbation prominently altered endothelial F-actin stress fiber formation. Together, these data support a model in which TLNRD1 preferentially modulates endothelial cytoskeletal remodeling largely independent of overt KLF2/4 transcriptional shifts, helping to contextualize previous discrepancies and refining our understanding of its role in vascular biology. - Source: PubMed
Publication date: 2026/06/01
Zhang YuchongQiu ShoujiHu ChengkaiFu WeiguoWang ChenjiWang Lixin - Recently, high-endurance ferroelectric HfO is highly desirable since the emerging of in-memory computing requires non-volatile memories not only to store data but also to execute computation, challenging writing/erasure switching reliability. Understanding and exploitation of the polarization fatigue diagram are crucial for improving endurance performances. Here, we show fatigue-resistant Sm:HfO thin films by modulating grain boundaries (GBs) in orientation-controllable orthorhombic phase. On GBs, orientation discontinuity raises energy levels of O 2p orbitals due to lattice distortion, which promote electron accumulation and yield a high-symmetry structural transform at the boundary, facilitating 90° switching of out-of-plane domains because of lowered switching barrier. Then the domains are frozen in plane by the charged GBs and polarization fatigue takes place. By eliminating GBs associated with phase transform, remarkably-improved fatigue resistance is achieved in uniform 180° switching, which exhibits the increase of fatigue-free endurance by 200 times to 2.0 × 10 cycles with, more importantly, a large field-cycling non-volatile polarization of ∼60 µC/cm, showing the state-of-the-art endurance performances in hafnium oxides. Roadmaps of the fatigue scenarios are given based on key roles of GBs in domain configurations and switching pathways. Our findings open a new perspective for fatigue studying and guide the material design of high-reliability hafnium oxide memories. - Source: PubMed
Publication date: 2026/07/03
Li JiufuLin ZehaoJing XixiangZheng WeijieWang ZhenJiang XinyuXu JiboZheng ChunyanLiu XiaohuiFu BeibeiHuang HaoliangWang Huan-HuaWu DiSong KepengCao TengfeiWen Zheng - Adults with unexplained neurologic presentations often undergo extensive evaluations without timely diagnosis. Evidence supporting the clinical utility of rapid whole-genome sequencing (rWGS) in hospitalized adult populations remains limited. We evaluated the diagnostic yield of rWGS in adults hospitalized for unexplained neurologic manifestations and assessed clinical predictors of a phenotype-concordant genetic diagnosis. - Source: PubMed
Publication date: 2026/06/16
Amanat ManToledano MichelSchimmenti Lisa APichurin Pavel NLanpher Brendan CDeyle David RTan Queenie K GIverson GabrielleCera AlannaGavrilova RalitzaDhamija Radhika