Recombinant Human Prolyl 3-hydroxylase 1 (LEPRE1)

中文名稱：

人P3H1重組蛋白
貨號(hào)：

CSB-YP657676HU
說(shuō)明書：

MSDS Datasheet
規(guī)格：
來(lái)源：

Yeast
其他：

中文名稱：

人P3H1重組蛋白
貨號(hào)：

CSB-EP657676HU-B
說(shuō)明書：

MSDS Datasheet
規(guī)格：
來(lái)源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

人P3H1重組蛋白
貨號(hào)：

CSB-BP657676HU
說(shuō)明書：

MSDS Datasheet
規(guī)格：
來(lái)源：

Baculovirus
其他：

中文名稱：

人P3H1重組蛋白
貨號(hào)：

CSB-MP657676HU
說(shuō)明書：

MSDS Datasheet
規(guī)格：
來(lái)源：

Mammalian cell
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

>85% (SDS-PAGE)
基因名：

P3H1
Uniprot No.：

Q32P28
別名：

GROS 1; GROS1; Growth suppressor 1; LEPRE 1; Lepre1; Leprecan 1; Leprecan; Leprecan-1; Leprecan1; Leucine proline enriched proteoglycan (leprecan) 1; Leucine proline enriched proteoglycan 1; Leucine- and proline-enriched proteoglycan 1; MGC117314; OI8; P3H1; P3H1_HUMAN; Prolyl 3 hydroxylase 1; Prolyl 3-hydroxylase 1
種屬：

Homo sapiens (Human)
蛋白長(zhǎng)度：

Full Length of Mature Protein
表達(dá)區(qū)域：

23-736
氨基酸序列

EVESEAGW GMVTPDLLFA EGTAAYARGD WPGVVLSMER ALRSRAALRA LRLRCRTQCA ADFPWELDPD WSPSPAQASG AAALRDLSFF GGLLRRAACL RRCLGPPAAH SLSEEMELEF RKRSPYNYLQ VAYFKINKLE KAVAAAHTFF VGNPEHMEMQ QNLDYYQTMS GVKEADFKDL ETQPHMQEFR LGVRLYSEEQ PQEAVPHLEA ALQEYFVAYE ECRALCEGPY DYDGYNYLEY NADLFQAITD HYIQVLNCKQ NCVTELASHP SREKPFEDFL PSHYNYLQFA YYNIGNYTQA VECAKTYLLF FPNDEVMNQN LAYYAAMLGE EHTRSIGPRE SAKEYRQRSL LEKELLFFAY DVFGIPFVDP DSWTPEEVIP KRLQEKQKSE RETAVRISQE IGNLMKEIET LVEEKTKESL DVSRLTREGG PLLYEGISLT MNSKLLNGSQ RVVMDGVISD HECQELQRLT NVAATSGDGY RGQTSPHTPN EKFYGVTVFK ALKLGQEGKV PLQSAHLYYN VTEKVRRIME SYFRLDTPLY FSYSHLVCRT AIEEVQAERK DDSHPVHVDN CILNAETLVC VKEPPAYTFR DYSAILYLNG DFDGGNFYFT ELDAKTVTAE VQPQCGRAVG FSSGTENPHG VKAVTRGQRC AIALWFTLDP RHSERDRVQA DDLVKMLFSP EEMDLSQEQP LDAQQGPPEP AQESLSGSES KPKDEL
蛋白標(biāo)簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產(chǎn)品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復(fù)溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲(chǔ)存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質(zhì)期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項(xiàng)：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.
基因功能參考文獻(xiàn)：
1. P3H1 mutation is associated with Non-Lethal Type VIII Osteogenesis Imperfecta. PMID: 27383115
2. This study enhances our knowledge about the mutational pattern of the LEPRE1, CRTAP, and PPIB genes. LEPRE1 should be the first gene analyzed in mutation detection studies in patients with recessive OI. PMID: 26634552
3. An additional function of the rough endoplasmic reticulum protein complex prolyl 3-hydroxylase 1.cartilage-associated protein.cyclophilin B: the CXXXC motif reveals disulfide isomerase activity in vitro. PMID: 24043621
4. This is the first report of a mutation in LEPRE1 that eliminates only the KDEL ER-retrieval sequence, whereas other functional domains remain intact PMID: 22615817
5. 0.4% of Mid-Atlantic African Americans and 1.48% West Africans carry mutation in LEPRE1 which causes lethal recessive osteogenesis imperfecta. PMID: 22281939
6. Mutation analyses were performed for COL1A1, COL1A2, CRTAP, and LEPRE1 in a cohort of 58 unrelated Chinese patients with osteogenesis imperfecta. PMID: 21667357
7. We report a large consanguineous Turkish family in which multiple individuals are affected with autosomal recessive lethal or severe osteogenesis imperfecta (OI) due to a novel homozygous LEPRE1 mutation PMID: 20946018
8. Null mutations in LEPRE1 and CRTAP cause severe recessive osteogenesis imperfecta. PMID: 19862557
9. CRTAP and P3H1 are mutually stabilized in the collagen prolyl 3-hydroxylation complex in endoplasmic reticulum. PMID: 19846465
10. Screening of 78 subjects diagnosed with osteogenesis imperfecta type II or III, identified three probands with mutations in CRTAP and 16 with mutations in LEPRE1. PMID: 18566967
11. Mutations in CRTAP and LEPRE1 are found in 3 patients with type II osteogenesis imperfecta. PMID: 18996919
12. Findings suggest that the 3-hydroxylation function of P3H1 is restricted to the 736AA splice form. PMID: 19088120
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相關(guān)疾病：

Osteogenesis imperfecta 8 (OI8)
亞細(xì)胞定位：

[Isoform 1]: Endoplasmic reticulum.; Secreted, extracellular space, extracellular matrix.
蛋白家族：

Leprecan family
數(shù)據(jù)庫(kù)鏈接：

HGNC: 19316

OMIM: 610339

KEGG: hsa:64175

STRING: 9606.ENSP00000296388

UniGene: Hs.720014