HGD Recombinant Monoclonal Antibody

說明書

中文名稱：

HGD重組抗體
貨號：

CSB-RA695552A0HU
規(guī)格：

￥1320
圖片：
IHC image of CSB-RA695552A0HU diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.

IHC image of CSB-RA695552A0HU diluted at 1:100 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品描述：

CSB-RA695552A0HU HGD重組單克隆抗體是針對人類尿黑酸1,2-雙加氧酶（Homogentisate 1,2-dioxygenase）研發(fā)的高特異性檢測工具，該酶是酪氨酸代謝途徑中的關(guān)鍵催化酶，其功能缺陷與遺傳性尿黑酸癥及相關(guān)骨關(guān)節(jié)病變密切相關(guān)。本抗體經(jīng)ELISA和免疫組化（IHC）嚴格驗證，在1:50-1:200稀釋范圍內(nèi)可清晰顯示組織樣本中HGD蛋白的定位分布，實驗數(shù)據(jù)顯示其在多種人類組織切片中呈現(xiàn)特異性染色模式，檢測結(jié)果與靶蛋白預(yù)期表達特征高度吻合，且不同批次產(chǎn)品具有良好穩(wěn)定性。適用于探究HGD在代謝性疾病發(fā)生發(fā)展中的作用機制，特別是在構(gòu)建酪氨酸代謝異常模型、骨關(guān)節(jié)退行性病變研究及病理樣本分析等領(lǐng)域具有重要應(yīng)用價值。該抗體為科研人員研究HGD蛋白表達調(diào)控及其在遺傳代謝缺陷中的病理生理意義提供了可靠工具。
Uniprot No.：

Q93099
基因名：

HGD
別名：

Homogentisate 1,2-dioxygenase (EC 1.13.11.5) (Homogentisate oxygenase) (Homogentisic acid oxidase) (Homogentisicase), HGD, HGO
反應(yīng)種屬：

Human
免疫原：

A synthesized peptide derived from human HGD
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

20D12
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

基因功能參考文獻：
1. Twelve novel HGD gene variants have been identified in 99 alkaptonuria patients affecting the bones. PMID: 25804398
2. A novel alkaptonuria mutation, c.87 + 1G > A, shows a significant founder effect and high prevalence in a nomadic Indian population. PMID: 24575791
3. study described two novel HGD mutations in a Chinese alkaptonuria family, the splicing mutation of IVS7 1G>C, a donor splice site of exon 7, and a missense mutation of F329C in exon 12 PMID: 23353776
4. Report mutations of the HGD gene in Jordanian alkaptonuria patients. PMID: 21437689
5. The observed increase of HGD expression in Alkaptonuria cells is probably due to a compensatory mechanism to overcome the almost null catalytic activity of the deficient enzyme PMID: 22105303
6. An update on molecular genetics of Alkaptonuria (AKU).(review) PMID: 21720873
7. Familiar ochronotic arthropathy is caused by a gene mutation of the homogentisic acid 1,2-dioxygenase (HGD) gene traced three hundred years in a Hungarian family. PMID: 20462779
8. A comprehensive mutation analysis of 93 patients enrolled in this study, as well as an extensive update of all previously published HGD mutations associated with alkaptonuria, is reported. PMID: 19862842
9. Turkish mutation shares an homogentisate 1,2-dioxygenase haplotype with the mutation found in Finland, Slovakia and India, suggesting that R58fs is an old alkaptonuria mutation that probably originated in central Asia PMID: 12872836
10. Four different mutations of the HGD gene were found in alkaptonuria and ochronotic arthropathy diagnoses. PMID: 16085442
11. A single nucleotide deletion located in exon 3 resulted in a frameshift mutation in HGD gene in family with alkaptonuria. PMID: 18945288
顯示更多

收起更多
相關(guān)疾病：

Alkaptonuria (AKU)
蛋白家族：

Homogentisate dioxygenase family
組織特異性：

Highest expression in the prostate, small intestine, colon, kidney and liver.
數(shù)據(jù)庫鏈接：

HGNC: 4892

OMIM: 203500

KEGG: hsa:3081

STRING: 9606.ENSP00000283871

UniGene: Hs.368254

Most popular with customers

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
WT1 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WDR5 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
VIM Recombinant Monoclonal Antibody

Applications: ELISA, IHC, IF

Species Reactivity: Human
VDR Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human