P3H2 Antibody

中文名稱：

P3H2兔多克隆抗體
貨號：

CSB-PA815555HA01HU
規格：

￥440
圖片：
Western Blot
Positive WB detected in: Mouse lung tissue, Mouse kidney tissue
All lanes: LEPREL1 antibody at 3μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 81, 61 kDa
Observed band size: 81, 61 kDa

Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA815555HA01HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA815555HA01HU at dilution of 1:100
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) P3H2 Polyclonal antibody
Uniprot No.：

Q8IVL5
基因名：

P3H2
別名：

4832416N06 antibody; AW553532 antibody; Leprecan like 1 antibody; Leprecan-like protein 1 antibody; Leprel1 antibody; MCVD antibody; MLAT4 antibody; Myxoid liposarcoma-associated protein 4 antibody; P3H2 antibody; P3H2_HUMAN antibody; Prolyl 3 hydroxylase 3 antibody; Prolyl 3-hydroxylase 2 antibody
宿主：

Rabbit
反應種屬：

Human, Mouse
免疫原：

Recombinant Human Prolyl 3-hydroxylase 2 protein (301-528AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，P3H2 Antibody (CSB-PA815555HA01HU)，的標記方式是Non-conjugated。對于P3H2 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA815555HB01HU	P3H2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA815555HC01HU	P3H2 Antibody, FITC conjugated
Biotin	CSB-PA815555HD01HU	P3H2 Antibody, Biotin conjugated	ELISA

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, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:2000-1:5000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) 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.

產品評價

靶點詳情

功能：

Prolyl 3-hydroxylase that catalyzes the post-translational formation of 3-hydroxyproline on collagens. Contributes to proline 3-hydroxylation of collagen COL4A1 and COL1A1 in tendons, the eye sclera and in the eye lens capsule. Has high activity with the type IV collagen COL4A1, and lower activity with COL1A1. Catalyzes hydroxylation of the first Pro in Gly-Pro-Hyp sequences where Hyp is 4-hydroxyproline. Has no activity on substrates that lack 4-hydroxyproline in the third position.
基因功能參考文獻：
1. Data identified an enhancer region within the TP63/LEPREL1 locus containing genetic variants associated with bladder cancer risk. PMID: 29956121
2. Recessive LEPREL1 mutations should be recognized as part of the differential diagnosis of lens subluxation. PMID: 25469533
3. LEPREL1 plays an important role in eye development and homozygous loss-of-function mutation of this gene can cause severely high myopia and early-onset cataract. PMID: 24172257
4. mutation in LRPAP1 is associated with high myopia. Further studies are expected to evaluate the pathogenicity of the variants in CTSH, LEPREL1, ZNF644, SLC39A5, and SCO2. PMID: 25525168
5. High myopia is caused by a mutation in LEPREL1, encoding prolyl 3-hydroxylase 2. PMID: 21885030
6. P3H2 has preferred substrate sequences among the classes of 3Hyp sites in clade A collagen chains PMID: 21757687
7. P3H2 is responsible for the hydroxylation of collagen IV, which has the highest 3-hydroxyproline content of all collagens. It is thus possible that P3H2 mutations may lead to a disease with changes in basement membranes. PMID: 18487197
8. The restriction of silencing in P3H2 to breast carcinomas, and its association with oestrogen-receptor-positive cases, suggests that P3H2 may be a breast-cancer-specific tumour suppressor. PMID: 19436308
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相關疾病：

Myopia, high, with cataract and vitreoretinal degeneration (MCVD)
亞細胞定位：

Endoplasmic reticulum. Sarcoplasmic reticulum. Golgi apparatus.
蛋白家族：

Leprecan family
組織特異性：

Expression localized to the epithelia of bile ducts and to the sacroplasm of heart muscle and skeletal muscle. In the pancreas, localized to a subpopulation of Langerhans islet cells and in the salivary gland, expressed in acinar cells (at protein level).
數據庫鏈接：

HGNC: 19317

OMIM: 610341

KEGG: hsa:55214

STRING: 9606.ENSP00000316881

UniGene: Hs.374191

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