Recombinant Mouse Phenylalanine-4-hydroxylase (Pah)

中文名稱：

小鼠Pah重組蛋白
貨號：

CSB-YP017396MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Pah重組蛋白
貨號：

CSB-EP017396MO-B
說明書：

MSDS Datasheet
規格：
來源：

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：

小鼠Pah重組蛋白
貨號：

CSB-BP017396MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Pah重組蛋白
貨號：

CSB-MP017396MO
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Pah
Uniprot No.：

P16331
別名：

Pah; Phenylalanine-4-hydroxylase; PAH; EC 1.14.16.1; Phe-4-monooxygenase
種屬：

Mus musculus (Mouse)
蛋白長度：

Full Length of Mature Protein
表達區域：

2-453
氨基酸序列

AAVVLENGV LSRKLSDFGQ ETSYIEDNSN QNGAVSLIFS LKEEVGALAK VLRLFEENEI NLTHIESRPS RLNKDEYEFF TYLDKRSKPV LGSIIKSLRN DIGATVHELS RDKEKNTVPW FPRTIQELDR FANQILSYGA ELDADHPGFK DPVYRARRKQ FADIAYNYRH GQPIPRVEYT EEERKTWGTV FRTLKALYKT HACYEHNHIF PLLEKYCGFR EDNIPQLEDV SQFLQTCTGF RLRPVAGLLS SRDFLGGLAF RVFHCTQYIR HGSKPMYTPE PDICHELLGH VPLFSDRSFA QFSQEIGLAS LGAPDEYIEK LATIYWFTVE FGLCKEGDSI KAYGAGLLSS FGELQYCLSD KPKLLPLELE KTACQEYTVT EFQPLYYVAE SFNDAKEKVR TFAATIPRPF SVRYDPYTQR VEVLDNTQQL KILADSINSE VGILCHALQK IKS
蛋白標簽：

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.
產品提供形式：

Lyophilized powder
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.
儲存條件：

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

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：

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.

產品評價

靶點詳情

功能：

Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.
基因功能參考文獻：
1. We propose that CNS monoamine deficiency may be the cause of the partially reversible adverse behavioral effects associated with chronic HPA in Pah(enu2) mice, but that phenylalanine-reducing treatments initiated during adulthood are unable to correct the neuropathology and attendant cognitive deficits that develop during juvenile life in late-treated Pah(enu2/enu2) mice PMID: 29331172
2. we assessed DNA methylation patterns in brain tissues using methylated DNA immunoprecipitation and paired end sequencing in adult PAH(enu2) animals maintained under either continuous dietary Phe restriction or chronic hyperphenylalaninemia. Heterozygous PAH(enu2/WT) litter mates served as controls for normal Phe exposure PMID: 26822703
3. These observations support the proposition that differences in phenylalanine hydroxylase activity underlie the variation in S-carboxymethyl-L-cysteine sulfoxidation and that no other enzyme is able to undertake this reaction. PMID: 26338263
4. A single injection of PAH-RBCs was able to decrease Phe levels by nearly 80% in normal mice PMID: 23867524
5. high levels of Phe, such as in phenylketonuria, are unlikely to directly induce brain hypomyelination, suggesting involvement of alternative mechanisms in this myelination defect PMID: 20151197
6. phenylalanine concentration regulates the activity of PAH in the hph-1 mouse and that this acts via a mechanism that includes phosphorylation of the PAH molecule PMID: 19560382
7. Tetrahydrobiopterin protects PAH activity in vivo. PMID: 15556637
8. low level of phenylalanine clearance was observed in mice expressing PAH and GTP-CH from the keratin 14 promoter PMID: 15946242
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相關疾病：

Mouse strains deficient in phenylalanine hydroxylase (Pah) were created as models of phenylketonuria (PKU).
蛋白家族：

Biopterin-dependent aromatic amino acid hydroxylase family
數據庫鏈接：

KEGG: mmu:18478

STRING: 10090.ENSMUSP00000020241

UniGene: Mm.263539