HOGA1 Antibody, Biotin conjugated

說明書

中文名稱：

HOGA1兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA771464LD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) HOGA1 Polyclonal antibody
Uniprot No.：

Q86XE5
基因名：

HOGA1
別名：

HOGA1 antibody; C10orf65 antibody; DHDPSL antibody; 4-hydroxy-2-oxoglutarate aldolase antibody; mitochondrial antibody; EC 4.1.3.16 antibody; Dihydrodipicolinate synthase-like antibody; DHDPS-like protein antibody; Probable 2-keto-4-hydroxyglutarate aldolase antibody; Probable KHG-aldolase antibody; Protein 569272 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human 4-hydroxy-2-oxoglutarate aldolase, mitochondrial protein (1-164AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

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

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA 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.

產品評價

靶點詳情

功能：

Catalyzes the final step in the metabolic pathway of hydroxyproline.
基因功能參考文獻：
1. Results show that HOGA1 harboring mutations found in primary hyperoxaluria is thermally unstable and targeted for proteolytic degradation leading to an absolute loss of function. PMID: 27096395
2. Among the seven patients identified with HOGA1 mutations the median onset of clinical symptoms was 1.8 years. Five patients initially presented with urolithiasis, and two other patients presented with urinary tract infection. PMID: 25972204
3. Our results strongly suggest HOGA1 as a major cause of PH, indicate a greater genetic heterogeneity of hyperoxaluria, and point to a favorable outcome of type III in the context of PH despite incomplete or absent biochemical remission PMID: 22781098
4. seven different mutations were identified in primary hyperoxaluria type 3 PMID: 22391140
5. Two crystal forms of DHDPSL were obtained, both of which diffracted X-rays to approximately 2.0 A resolution PMID: 22232173
6. hHOGA utilizes a type I aldolase reaction mechanism, but employs novel residue interactions for substrate binding PMID: 21998747
7. Detection of HOGA1 variants in idiopathic calcium oxalate urolithiasis also suggests HOGA1 may be a predisposing factor for this condition PMID: 21896830
8. DHDPSL is the gene encoding 4-hydroxy-2-oxoglutarate aldolase, catalyzing the final step in the metabolic pathway of hydroxyproline. PMID: 20797690
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相關疾病：

Hyperoxaluria primary 3 (HP3)
亞細胞定位：

Mitochondrion.
蛋白家族：

DapA family
數據庫鏈接：

HGNC: 25155

OMIM: 613597

KEGG: hsa:112817

STRING: 9606.ENSP00000359680

UniGene: Hs.180346

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