HPA023597
抗-LRBA 兔抗
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab2
别名:
抗- Beige样蛋白, 抗-CDC4样蛋白, 抗-脂多糖反应和浅褐色样锚蛋白
产品名称
抗-LRBA 兔抗, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab2
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
enhanced validation
RNAi knockdown
Learn more about Antibody Enhanced Validation
technique(s)
immunohistochemistry: 1:1000- 1:2500
immunogen sequence
GGWRVWVDTLSITHSKVTFEIHKENLANIFREQQGKVDEEIGLCSSTSVQAASGIRRDINVSVGSQQPDTKDSPVCPHFTTNGNENSSIEKTSSLESASNIELQTTNTSYEEMKAEQENQELPDEGTLEETL
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... LRBA(987)
Application
成功使用该抗体的应用以及相关的同行评审论文如下所示。
蛋白质免疫印迹分析（1篇论文）
蛋白质免疫印迹分析（1篇论文）
抗-LRBA兔抗可用于western blotting。
由Atlas Antibodies提供技术支持的所有Prestige Antibodies®抗体均由人类蛋白质图集（HPA）项目开发和验证。每种抗体都通过针对数百种正常和疾病组织的免疫组织化学进行测试。通过单击图像库链接，可以在人类蛋白质图谱（HPA）站点上查看这些图像。我们还提供Prestige Antibodies® 抗体的实验方案和其他有用信息。
由Atlas Antibodies提供技术支持的所有Prestige Antibodies®抗体均由人类蛋白质图集（HPA）项目开发和验证。每种抗体都通过针对数百种正常和疾病组织的免疫组织化学进行测试。通过单击图像库链接，可以在人类蛋白质图谱（HPA）站点上查看这些图像。我们还提供Prestige Antibodies® 抗体的实验方案和其他有用信息。
Biochem/physiol Actions
LRBA 基因（lipopolysaccharide-responsive and beige-like anchor protein）编码的蛋白参与配体活化受体的内噬。LRBA中的突变与炎症性肠病（IBD）样症状、自身免疫性淋巴增生综合症样疾病和IPEX（免疫失调、多内分泌病、肠病、X连锁）综合征有关。
Disclaimer
除非我们的产品目录或产品附带的其他公司文档另有说明，否则我们的产品仅供研究使用，不得用于任何其他目的，包括但不限于未经授权的商业用途、体外诊断用途、离体或体内治疗用途或任何类型的消费或应用于人类或动物。
Features and Benefits
Prestige抗体®是经过高度表征和广泛验证的抗体，同时还有一个优点是其每个靶标的所有可用表征数据都可以通过位于此页面顶部产品名称下方的人类蛋白质图谱门户进行访问。Prestige抗体对其他蛋白质的独特性和低交叉反应性®是通过严密的抗原区域选择、亲和纯化和严格的选择来实现的。针对每一个Prestige Antibody都存在有对应的Prestige抗原对照，并可在链接区域内找到。
每个Prestige Antibody都是按照以下方法进行测试的：
每个Prestige Antibody都是按照以下方法进行测试的：
- 44例正常人类组织以及20例最常见癌症类型组织的IHC组织阵列。
- 364个人类重组蛋白片段的蛋白阵列。
General description
LRBA 基因（lipopolysaccharide-responsive and beige-like anchor protein）定位于人类染色体4q31.3。它属于BEACH （beige and chediak-higashi）-WD40 (β-转导蛋白) 蛋白家族。LRBA 广泛表达。该蛋白位于细胞质。通过激活B细胞，LRBA 转移到高尔基体反面的膜、溶酶体、内质网和细胞表面。
Immunogen
脂多糖反应性和beige样锚蛋白重组蛋白表位标记标签（PrEST）
Other Notes
相应抗原APREST74668
Physical form
溶于磷酸盐缓冲盐水，pH 7.2，含有40%甘油和0.02%叠氮化钠
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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存储类别
10 - Combustible liquids
wgk
WGK 1
法规信息
常规特殊物品
常规特殊物品
此项目有
Eriko Totsune et al.
Frontiers in immunology, 12, 677572-677572 (2021-04-30)
Lipopolysaccharide-responsive beige-like anchor (LRBA) deficiency is a subtype of common variable immune deficiency (CVID). Numerous case reports and cohort studies have described a broad spectrum of clinical manifestations and variable disease phenotypes, including immune dysregulation, enteropathy, and recurrent infections. Although
Wen-Juan Tang et al.
World journal of clinical cases, 9(21), 5873-5888 (2021-08-10)
Patients with lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency have a variety of clinical symptoms, but there is no apparent genotype-phenotype correlation, and patients carrying the same mutations may have different phenotypes. Therefore, it is not easy for doctors to
Fabienne Charbit-Henrion et al.
Journal of Crohn's & colitis (2018-05-23)
An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases (VEO-IBD). The present study aimed at defining how next-generation sequencing (NGS) methods can be used to improve identification of known
Shahrzad Bakhtiar et al.
Frontiers in pediatrics, 4, 98-98 (2016-09-30)
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a
Atypical Manifestation of LPS-Responsive Beige-Like Anchor Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency.
Bakhtiar S
Frontiers in pediatrics, 4 (2016)
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