Anti-Ext1抗体(ab126305)
Key features and details
- Rabbit polyclonal to Ext1
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG
概述
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产品名称
Anti-Ext1抗体 -
描述
兔多克隆抗体to Ext1 -
宿主
Rabbit -
经测试应用
适用于: IHC-Pmore details -
种属反应性
与反应: Human -
免疫原
antigen sequence, corresponding to amino acids 205-335 of Human Ext1.
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阳性对照
- IHC-P: Human rectum, placenta, liver and prostate tissue.
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 59% PBS -
Concentration information loading... -
纯度
Immunogen affinity purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab126305于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。
| 应用 | Ab评论 | 说明 |
|---|---|---|
| IHC-P | (1) |
1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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| 说明 |
|---|
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IHC-P
1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
靶标
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功能
Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor. -
组织特异性
Ubiquitous. -
通路
Protein modification; protein glycosylation. -
疾病相关
Defects in EXT1 are a cause of hereditary multiple exostoses type 1 (EXT1) [MIM:133700]. EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event.
Defects in EXT1 are a cause of tricho-rhino-phalangeal syndrome type 2 (TRPS2) [MIM:150230]. A syndrome that combines the clinical features of trichorhinophalangeal syndrome type 1 and multiple exostoses type 1. Affected individuals manifest multiple dysmorphic facial features including large, laterally protruding ears, a bulbous nose, an elongated upper lip, as well as sparse scalp hair, winged scapulae, multiple cartilaginous exostoses, redundant skin, and mental retardation. Note=A chromosomal aberration resulting in the loss of functional copies of TRPS1 and EXT1 has been found in TRPS2 patients.
Defects in EXT1 are a cause of chondrosarcoma (CHDSA) [MIM:215300]. It is a malignant neoplasm derived from cartilage cells. Chondrosarcomas range from slow-growing non-metastasizing lesions to highly aggressive metastasizing sarcomas. -
序列相似性
Belongs to the glycosyltransferase 47 family. -
细胞定位
Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus. - Information by UniProt
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数据库链接
- Entrez Gene: 2131 Human
- Omim: 608177 Human
- SwissProt: Q16394 Human
- Unigene: 492618 Human
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别名
- 4-alpha-N-acetylglucosaminyltransferase antibody
- exostoses (multiple) 1 antibody
- Exostosin 1 antibody
see all
图片
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ext1 antibody (ab126305)
Immunohistochemical analysis of human rectum tissue labeling Ext1 in the cytoplasm of glandular cells with ab126305 at a 1/50 dilution.
Performed heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ext1 antibody (ab126305)Immunohistochemical analysis of human placenta tissue labeling Ext1 in the cytoplasm of trophoblastic cells with ab126305 at a 1/50 dilution.
Performed heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ext1 antibody (ab126305)Immunohistochemical analysis of human liver tissue labeling Ext1 in the cytoplasm of hepatocytes with ab126305 at a 1/50 dilution.
Performed heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ext1 antibody (ab126305)Immunohistochemical analysis of human prostate tissue labeling Ext1 in the cytoplasm of smooth muscle cells with ab126305 at a 1/50 dilution.
Performed heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
数据表及文件
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SDS download
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Datasheet download
文献 (6)
ab126305 被引用在 6 文献中.
- Duplancic R & Kero D Novel approach for quantification of multiple immunofluorescent signals using histograms and 2D plot profiling of whole-section panoramic images. Sci Rep 11:8619 (2021). PubMed: 33883639
- Kerselidou D et al. Alternative glycosylation controls endoplasmic reticulum dynamics and tubular extension in mammalian cells. Sci Adv 7:N/A (2021). PubMed: 33962942
- Guo X et al. A novel splice mutation induces exon skipping of the EXT1 gene in patients with hereditary multiple exostoses. Int J Oncol 54:859-868 (2019). PubMed: 30664192
- Duplancic R et al. Syndecans and Enzymes for Heparan Sulfate Biosynthesis and Modification Differentially Correlate With Presence of Inflammatory Infiltrate in Periodontitis. Front Physiol 10:1248 (2019). PubMed: 31611818
- Kero D et al. Syndecans and Enzymes Involved in Heparan Sulfate Biosynthesis and Degradation Are Differentially Expressed During Human Odontogenesis. Front Physiol 9:732 (2018). IHC-P ; Human . PubMed: 29962964
- Zhang F et al. Exome sequencing and functional analysis identifies a novel mutation in EXT1 gene that causes multiple osteochondromas. PLoS One 8:e72316 (2013). IHC-P ; Human . PubMed: 24009674