PEX7 Antibody

說明書

中文名稱：

PEX7兔多克隆抗體
貨號：

CSB-PA017808GA01HU
規(guī)格：

￥3,900
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

Uniprot No.：

O00628
基因名：

PEX7
別名：

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

Rabbit
反應(yīng)種屬：

Human,Mouse,Rat
免疫原：

Human PEX7
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

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

PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA,WB
Protocols：

ELISA Protocol
Western Blotting (WB) 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(chǎn)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(xiàn)：
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(guān)疾病：

Peroxisome biogenesis disorder complementation group 11 (PBD-CG11); Rhizomelic chondrodysplasia punctata 1 (RCDP1); Peroxisome biogenesis disorder 9B (PBD9B)
亞細(xì)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(shù)據(jù)庫鏈接：

HGNC: 8860

OMIM: 215100

KEGG: hsa:5191

STRING: 9606.ENSP00000315680

UniGene: Hs.280932

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