Recombinant Human Peroxisomal targeting signal 2 receptor (PEX7)

中文名稱：

人PEX7重組蛋白
貨號：

CSB-YP017808HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人PEX7重組蛋白
貨號：

CSB-EP017808HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人PEX7重組蛋白
貨號：

CSB-EP017808HU-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：

人PEX7重組蛋白
貨號：

CSB-BP017808HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人PEX7重組蛋白
貨號：

CSB-MP017808HU
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：

PEX7
Uniprot No.：

O00628
別名：

PBD9B; PCDP1; Peroxin 7; Peroxin-7; Peroxisomal PTS2 receptor; Peroxisomal targeting signal 2 receptor; Peroxisome biogenesis factor 7; Peroxisome targeting signal 2 receptor; PEX7; PEX7 protein; PEX7_HUMAN; PTS2 receptor; PTS2R; RCDP1; RD
種屬：

Homo sapiens (Human)
蛋白長度：

Full length protein
表達區域：

1-323
氨基酸序列

MSAVCGGAAR MLRTPGRHGY AAEFSPYLPG RLACATAQHY GIAGCGTLLI LDPDEAGLRL FRSFDWNDGL FDVTWSENNE HVLITCSGDG SLQLWDTAKA AGPLQVYKEH AQEVYSVDWS QTRGEQLVVS GSWDQTVKLW DPTVGKSLCT FRGHESIIYS TIWSPHIPGC FASASGDQTL RIWDVKAAGV RIVIPAHQAE ILSCDWCKYN ENLLVTGAVD CSLRGWDLRN VRQPVFELLG HTYAIRRVKF SPFHASVLAS CSYDFTVRFW NFSKPDSLLE TVEHHTEFTC GLDFSLQSPT QVADCSWDET IKIYDPACLT IPA
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：

Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
基因功能參考文獻：
1. This revealed a marked plasmalogen deficiency and a deficient fatty acid alpha-oxidation in the IHH cells, due to a defect of PEX7, a cytosolic receptor protein required for peroxisomal import of a subset of peroxisomal proteins. PMID: 28013369
2. our data suggest that insertion of the trimeric PEX5-PEX7-PTS2 protein complex into the DTM is probably accompanied by conformational alterations in PEX5 to allow release of the PTS2 protein into the organelle matrix PMID: 26138649
3. Mutation in the PEX7 Gene is associated with Rhizomelic Chondrodysplasia Punctata Type 1. PMID: 25800479
4. the sequential formation of a highly stable trimeric complex involving cargo protein, PEX7 and PEX5L stabilizes cargo binding and is a prerequisite for PTS2-mediated peroxisomal import. PMID: 25538232
5. dysfunctional Pex7p, including mutants from RCDP patients, is degraded by a ubiquitin-dependent proteasomal pathway involving the CRL4A (Cullin4A-RING ubiquitin ligase) complex. PMID: 24989250
6. Export of peroxisomal PEX7 back into the cytosol requires export of PEX5. PMID: 24865970
7. This results of this studt revealed the association of 2 single nucleotide polymorphisms and 1 haplotype with autism spectrum disorder (P < .05). PMID: 22378669
8. Structural requirements for interaction of peroxisomal targeting signal 2 and its receptor PEX7. PMID: 22057399
9. Mutations in PEX7 gene is associated with Rhizomelic chondrodysplasia punctata. PMID: 20145307
10. mutational spectrum in the PEX7 gene of 78 patients (including five pairs of sibs) clinically and biochemically diagnosed with RCDP type I PMID: 11781871
11. Functional studies on human Pex7p: subcellular localization and interaction with proteins containing a peroxisome-targeting signal type 2 and other peroxins. PMID: 11931631
12. The residual activity of mutant Pex7 protein in rhizomelic chondrodysplasia punctata patients and reduced amounts of normal Pex7 are associated with milder and variant phenotypes. PMID: 12325024
13. This gene codes for the peroxin 7 receptor protein required for peroxisomal import of proteins containing a peroxisomal targeting signal type 2. Mutations may result in a broad clinical spectrum of Refsum disease. PMID: 12522768
14. Identification of PEX7 as the second gene involved in Refsum disease. PMID: 14713215
15. Missense mutations, sequence duplications and deletions in PRX7 in 3 patients with the Refsum disease phenotypes. PMID: 14974078
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相關疾病：

Peroxisome biogenesis disorder complementation group 11 (PBD-CG11); Rhizomelic chondrodysplasia punctata 1 (RCDP1); Peroxisome biogenesis disorder 9B (PBD9B)
亞細胞定位：

Peroxisome. Cytoplasm.
蛋白家族：

WD repeat peroxin-7 family
組織特異性：

Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
數據庫鏈接：

HGNC: 8860

OMIM: 215100

KEGG: hsa:5191

STRING: 9606.ENSP00000315680

UniGene: Hs.280932