Mucin

黏蛋白
  • 文章类型: Case Reports
    This study describes a unique case of single mucin-rich brain metastasis in a patient with breast cancer, mimicking the T2-fluid attenuation inversion recovery (FLAIR) mismatch sign and masquerading as an isocitrate dehydrogenase-mutant astrocytoma. This case highlights the importance of considering mucin-rich lesions in the differential diagnosis of intracranial tumors exhibiting T2-FLAIR mismatch. Clinicians must recognize the potential convergence in imaging characteristics between these metastases and gliomas to guarantee prompt and accurate patient care.
    T2-fluid attenuation inversion recovery (이하 FLAIR) mismatch sign은 isocitrate dehydrogenase-mutant 성상세포종을 시사하는 영상 소견으로 알려져 있다. 이 증례 보고에서는 유방암 환자의 뇌에 생긴 점액성 뇌전이암이 T2-FLAIR mismatch sign처럼 보이는 사례를 소개한다. 특히 비조영증강 MRI에서 T2-FLAIR mismatch sign을 보이는 경우, 성상세포종 뿐만 아니라 뇌전이암을 감별진단에 염두에 두어야 한다.
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  • 文章类型: Case Reports
    肢端持续性丘疹性黏液病(APPM)是一种罕见的特发性局部扁平苔藓粘液症亚型。迄今为止,全球报告的APPM病例不到41例,然而,几乎所有患者年龄均大于18岁.本文首次报道了一个7岁的孩子。
    一名7岁男孩因右手食指中段的桡侧有一个孤立的皮肤色丘疹入院。患者想知道确切的诊断并将其移除,因为中段的屈曲运动已受到影响。因此,进行了手术。从右食指中段的丘疹放射状侧获得的活检标本的组织病理学检查显示,粘蛋白在乳头和中皮层中有局灶性和界限明确的沉积。沉积物从未深入网状真皮。粘蛋白避开了乳头状真皮的表皮下区域。Alcian蓝色污渍可以突出粘蛋白。丘疹在组织学上被诊断为APPM,并通过手术切除。手术后伤口逐渐愈合,没有明显的复发,到目前为止,在随访期间观察到疤痕或其他不适。
    据我们所知,这是罕见的儿童APPM表现为影响中段屈曲运动的孤立丘疹。因为它是一种罕见的疾病,我们报道该病例,为今后对APPM的诊断和发病机制的研究做出贡献。
    UNASSIGNED: Acral persistent papular mucinosis (APPM) is a rare idiopathic subtype of localized lichen myxedematosus. To date, there have been less than 41 APPM cases reported worldwide, however, almost all patients were older than 18 years of age. A 7-year-old child was first reported in this paper.
    UNASSIGNED: A 7-year-old boy was admitted to our hospital with a solitary skin-colored papule on the radial side of the middle segment of his right index finger. The patient wanted to know the exact diagnosis and remove it because the flexion movement of the middle segment had been affected. Thus, a surgery was performed. Histopathological examination of a biopsy specimen obtained from the papule on the radial side of the middle segment of his right index finger showed a focal and well-circumscribed deposit of mucin in the papillary and middermis. The deposit never extended deeply into the reticular dermis. Mucin spared a subepidermal area in the papillary dermis. Alcian blue stains can highlight the mucin. The papule was histologically diagnosed as an APPM and excised surgically. The wound gradually healed after the operation, and no obvious recurrence, scar or other discomfort was observed during follow-up so far.
    UNASSIGNED: To the best of our knowledge, this is the rare case of a child APPM presenting as a solitary papule affecting the flexion movement of the middle segment. Since it is a rare disease, we report this case to contribute to future research on the diagnosis and pathogenesis of APPM.
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  • 文章类型: Journal Article
    基于纳米颗粒的处理对于水产养殖至关重要。在这种情况下,由于壳聚糖的性质,已经提出了基于壳聚糖的纳米颗粒,其中包括粘膜粘附性。然而,壳聚糖的关键参数,如乙酰化程度和分子量,尽管它们似乎对壳聚糖基纳米颗粒的性质有影响,但在现有文献中通常被低估。在这次系统审查中,对壳聚糖纳米粒作为硬骨鱼粘膜疫苗的免疫调节剂能力进行了评价,特别关注壳聚糖的特性。四个数据库用于文献检索,产生486个文档,其中14人符合纳入标准。只有21%的可用研究报告了适当的壳聚糖特性,应在未来的工作中进行改进,以生成可重复的数据和有价值的信息。据我们所知,这项工作首次客观地进行了比较,通过量化每一项研究中应用的壳聚糖的毫克数/克鱼,壳聚糖纳米颗粒的制备和应用于鱼类的剂量,以及治疗对鱼类免疫状态的影响。
    Nanoparticles-based treatments is of utmost importance for aquaculture. In this scenario, chitosan-based nanoparticles have been proposed due to the properties of chitosan, which include mucoadhesiveness. Nevertheless, pivotal parameters of chitosan, such as degree of acetylation and molecular weight, are commonly underestimated in the available literature despite the influence they seem to have on the properties of chitosan-based nanoparticles. In this systematic review, the immunomodulator capacity of chitosan nanoparticles used as mucosal vaccines on teleost fish has been evaluated paying special attention to the chitosan properties. Four databases were used for literature search, yielding 486 documents, from which 14 meet the inclusion criteria. Only 21% of the available studies reported properly chitosan properties, which should be improved in future works to generate reproducible data as well as valuable information. To the best of our knowledge, this work objectively compares for the first time, by quantifying the mg of chitosan/g of fish applied in each study, the chitosan nanoparticle preparation and doses applied to fish, as well as the effects of the treatments applied on fish immune status.
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  • 文章类型: Case Reports
    Pseudomyxoma peritonei (PMP) is a rare and uncommon condition, characterized by the presence of mucinous ascites in the abdominal cavity. The most common cause of PMP is mucinous adenocarcinoma of the appendix, followed by neoplasms of the ovary, endocervix, fallopian tube, alimentary organs, urachus, urinary bladder, lung, mucinous cyst of the spleen, and breast. Herein, we report a case of a 64-year-old postmenopausal woman (gravida 2, para 2) who presented at the department of gynecology with a short history of nausea and abdominal distention. Abdominal and vaginal ultrasonography showed a large amount of free fluid in the pelvis with hyperechoic echogenicity and right pelvic tumor with mixed echogenicity. Computed tomography demonstrated the presence of a heterogeneous, hypodense mass, without contrast enhancement, located on the right side of the pelvis, near the right ovary. Laparotomy was performed. Revision of the abdominal cavity revealed a large amount of yellow gelatinous mucinous ascites - approximately 1.5 l. A tumor (6 x 7 cm in diameter), arising from the appendix and located in the pouch of Douglas near the right ovary, was observed. Histopathology examination revealed poorly differentiated mucinous appendiceal adenocarcinoma, comprising up to 50% signet ring cells. Gastrointestinal tumors such as appendiceal neoplasms combined with PMP may mimic ovarian carcinomas. Computed tomography, abdominal/vaginal ultrasonography and tumor marker levels (carcino-embryonic antigen, carbohydrate antigen 19.9, carbohydrate antigen Ca-125) may establish the diagnosis. A differential diagnosis with appendiceal tumors should be considered for patients with right pelvic masses.
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  • 文章类型: Journal Article
    粘蛋白是一类糖基化蛋白,是粘液的主要成分,在人体保护性粘膜屏障的调节中起着动态作用。溃疡性结肠炎(UC)是一种炎症性肠病(IBD),其特征是大肠内层持续发炎。在本系统综述中,我们分析了目前可用的数据,以确定UC患者结肠粘膜粘蛋白活性是否存在改变.进行了数据库搜索,以确定1990年至2020年之间发表的评估粘蛋白在UC患者队列中的作用的研究。切除活检标本进行分析,纳入对照组进行比较.最初确定了5497篇文章,并系统地选择了这14项研究进行分析,通过引文链接确定了另外2篇文章。因此,对16项研究进行了严格审查。这些研究中的13项评估了MUC2在UC中的作用,大多数文章表明MUC2结构或合成的改变对结肠粘膜有影响。尽管关于MUC2表达的结果相互矛盾。这篇综述强调了进一步研究的重要性,以增强我们对UC粘蛋白调节的理解,并总结了可能为未来研究提供信息的数据。
    Mucins are a family of glycosylated proteins which are the primary constituents of mucus and play a dynamic role in the regulation of the protective mucosal barriers throughout the human body. Ulcerative colitis (UC) is an Inflammatory Bowel Disease (IBD) characterised by continuous inflammation of the inner layer of the large intestine, and in this systematic review we analyse currently available data to determine whether alterations exist in mucin activity in the colonic mucosa of UC patients. Database searches were conducted to identify studies published between 1990 and 2020 that assess the role of mucins in cohorts of UC patients, where biopsy specimens were resected for analysis and control groups were included for comparison. 5497 articles were initially identified and of these 14 studies were systematically selected for analysis, a further 2 articles were identified through citation chaining. Therefore, 16 studies were critically reviewed. 13 of these studies assessed the role of MUC2 in UC and the majority of articles indicated that alterations in MUC2 structure or synthesis had an impact on the colonic mucosa, although conflicting results were presented regarding MUC2 expression. This review highlights the importance of further research to enhance our understanding of mucin regulation in UC and summarises data that may inform future studies.
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  • 文章类型: Journal Article
    Mucinous colorectal cancer (CRC) is estimated to occur in approximately 10-15% of CRC cases and is characterized by abundant extracellular mucin. Mucinous CRC is frequently associated with resistance to apoptosis. Inferior prognosis is observed in mucinous CRC, particularly in rectal cancer and metastatic cases. Mucins are heavily glycosylated secretory or transmembrane proteins that participate in protection of the colonic epithelium. MUC2 overexpression is a hallmark of mucinous CRCs. Mucinous CRC is associated with KRAS and BRAF mutation, microsatellite instability and the CpG island methylator phenotype. Mutations of the APC gene and p53 mutations which are characteristic non-mucinous colorectal adenocarcinoma are less common in mucinous CRC. Both physical and anti-apoptotic properties of mucin provide mechanisms for resistance to cell death. Mucin glycoproteins are associated with decreased expression of pro-apoptotic proteins, increased expression of anti-apoptotic proteins and increased cell survival signaling. The role for BCL-2 proteins, including BCL-XL, in preventing apoptosis in mucinous CRC has been explored to a limited extent. Additional mechanisms opposing cell death include altered death receptor expression and altered mutation rates in genes responsible for chemotherapy resistance. The roles of alternate cell death programs including necroptosis and pyroptosis are not well understood in mucinous CRC. While the presence of MUC2 is associated with an immunosuppressive environment, the tumor immune environment of mucinous CRC and the role of immune-mediated tumor cell death likewise require further investigation. Improved understanding of cell death mechanisms in mucinous CRC may allow modification of currently used regimens and facilitate targeted treatment.
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  • 文章类型: Systematic Review
    BACKGROUND: Mucus protects the epithelium against invaders and toxic materials. Sticky and thick mucus is characteristic of CF.
    OBJECTIVE: The aim of this systematic review is to characterize the specific mucins secreted in the lung and intestinal tract of CF patients.
    METHODS: A systematic literature search was conducted up to December 31, 2019. The following terms were used: \"cystic fibrosis\" AND \"mucin.\" Case-control studies comparing mucin expression in CF patients to healthy controls were included.
    RESULTS: We found 741 eligible studies, 694 studies were rejected because they were performed in animals and not in full text, and 32 studies were excluded being editorials, duplications, review articles, meta-analysis, or not in English. Fifteen studies were eligible for our study, including 150 CF patients compared to 82 healthy controls, all fulfilled the inclusion criteria. The main mucin types expressed in the sinus submucosal glands, sputum, tracheobronchial surface epithelium, and lung submucosal glands were MUC5AC and MUC5B. Increase in the number of sinusoidal submucosal glands and expression of MUC5B was found in CF patients, but no such difference from healthy controls was found for the number of goblet cells in the surface epithelium nor in the expression of -MUC5AC. The opposite was found in the tracheobronchial surface epithelium and in the lungs.
    CONCLUSIONS: Increased expression of MUC5AC in the surface epithelium and of MUC5B in the subepithelial glands may be the result of higher secretion rate of mucin into the lumen of the respiratory tract, causing mucus plaque, infection, and inflammation.
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  • 文章类型: Journal Article
    目的:腹膜假黏液瘤(PMP)是一种罕见的临床恶性肿瘤综合征,其特征是大量黏液性腹水在腹膜腔内不可控制地积聚,导致“果冻肚子”。肿瘤进展和粘蛋白高分泌的机制仍然是未知的。但是GNAS突变是一个有前途的因素。本文旨在系统地总结GNAS的生物学背景和变异特征,以及GNAS突变对粘蛋白表达的影响,肿瘤细胞增殖,临床病理特征,和PMP的预后。
    方法:使用NCBIPubMed数据库(英文)和WANFANGDATA(中文)进行文献检索。NCBI基因和蛋白质数据库,Ensembl基因组浏览器,COSMIC,UniProt,和RCSBPDB数据库用于基因和蛋白质审查。
    结果:GNAS编码鸟嘌呤核苷酸结合蛋白α亚基(Gsα)。PMP中GNAS突变的突变位点相对稳定,通常在Chr20:57,484,420(碱基对:C-G)和Chr20:57,484,421(碱基对:G-C)。典型的GNAS突变导致Gsα中GTP酶活性降低,导致无法水解GTP并释放磷酸,最终GTP与Gsα的连续结合。激活的Gsα可通过刺激cAMP-PKA信号通路持续促进粘蛋白分泌,这是导致PMP粘蛋白分泌增加的可能机制。
    结论:GNAS突变是PMP中最重要的分子生物学特征之一,具有促进粘蛋白高分泌的主要功能。
    OBJECTIVE: Pseudomyxoma peritonei (PMP) is a rare clinical malignancy syndrome characterized by the uncontrollable accumulation of copious mucinous ascites in the peritoneal cavity, resulting in \"jelly belly\". The mechanism of tumor progression and mucin hypersecretion remains largely unknown, but GNAS mutation is a promising contributor. This review is to systemically summarize the biological background and variant features of GNAS, as well as the impacts of GNAS mutations on mucin expression, tumor cell proliferation, clinical-pathological characteristics, and prognosis of PMP.
    METHODS: NCBI PubMed database (in English) and WAN FANG DATA (in Chinese) were used for literature search. And NCBI Gene and Protein databases, Ensembl Genome Browser, COSMIC, UniProt, and RCSB PDB database were used for gene and protein review.
    RESULTS: GNAS encodes guanine nucleotide-binding protein α subunit (Gsα). The mutation sites of GNAS mutation in PMP are relatively stable, usually at Chr20: 57,484,420 (base pair: C-G) and Chr20: 57,484,421 (base pair: G-C). Typical GNAS mutation results in the reduction of GTP enzyme activity in Gsα, causing failure to hydrolyze GTP and release phosphoric acid, and eventually the continuous binding of GTP to Gsα. The activated Gsα could thus continuously promote mucin secretion through stimulating the cAMP-PKA signaling pathway, which is a possible mechanism leading to elevated mucin secretion in PMP.
    CONCLUSIONS: GNAS mutation is one of the most important molecular biological features in PMP, with major functions to promote mucin hypersecretion.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    The literature on the prognostic relevance of signet-ring cell (SRC) histology in gastric cancer (GC) is controversial which is most likely related to inconsistent SRC classification based on haematoxylin-eosin staining. We hypothesised that mucin stains can consistently identify SRC-GC and predict GC patient outcome.
    We performed a comprehensive literature review on mucin stains in SRC-GC and characterised the mucin expression in 851 Caucasian GC and 410 Asian GC using Alcian Blue (AB)-Periodic Acid-Schiff (PAS), MUC2 (intestinal-type mucin), and MUC5AC (gastric-type mucin). The relationship between mucin expression and histological phenotype [poorly cohesive (PC) including proportion of SRCs, non-poorly cohesive (non-PC), or mucinous (MC)], clinicopathological variables, and patient outcome was analysed.
    Depending on mucin expression and cut-offs, the positivity rates of SRC-GC reported in the literature varied from 6 to 100%. Patients with MUC2 positive SRC-GC or SRC-GC with (gastro)intestinal phenotype had poorest outcome. In our cohort study, PC with ≥ 10% SRCs expressed more frequently MUC2, MUC5AC, and ABPAS (p < 0.001, p = 0.004 and p < 0.001, respectively). Caucasians with AB positive GC or combined ABPAS-MUC2 positive and MUC5AC negative had poorest outcome (all p = 0.002). This association was not seen in Asian patients.
    This is the first study to suggest that mucin stains do not help to differentiate between SRC-GC and non-SRC-GC. However, mucin stains appear to be able to identify GC patients with different outcome. To our surprise, the relationship between outcome and mucin expression seems to differ between Caucasian and Asian GC patients which warrants further investigations.
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