PEX1 Antibody, FITC conjugated

說明書

中文名稱：

PEX1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA017793LC01HU
規格：

￥880
其他：

產品詳情
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) PEX1 Polyclonal antibody
Uniprot No.：

O43933
基因名：

PEX1
別名：

Highly similar to ATPase [H.sapiens] antibody; Peroxin 1 antibody; Peroxin-1 antibody; peroxin1 antibody; Peroxisome biogenesis disorder protein 1 antibody; Peroxisome biogenesis factor 1 antibody; PEX1 antibody; PEX1_HUMAN antibody; ZWS1 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Peroxisome biogenesis factor 1 protein (927-1283AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
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
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：

Required for stability of PEX5 and protein import into the peroxisome matrix. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.
基因功能參考文獻：
1. heterozygous mutations in the PEX1 gene in two Chinese newborns with Zellweger syndrome PMID: 28432012
2. Major finding is linking peroxisome biogenesis factor 1 (PEX1) to obesity phenotypes, a novel mechanism of peroxisomal biogenesis and metabolism underlying the development of childhood obesity. PMID: 28508493
3. A combination of a known missense and novel frameshift variant in PEX1 identified in a family with Heimler syndrome. PMID: 27302843
4. As standard biochemical screening of blood for evidence of a peroxisomal disorder did not provide a diagnosis in the individuals with Heimler syndrome, patients with sensorineural hearing lossand retinal pigmentation should have mutation analysis of PEX1 and PEX6 genes. PMID: 27633571
5. Our structural data suggest that the tilting of a central segment of a Pex1-Pex6 pair is responsible for polypeptide movement. PMID: 26476099
6. Mutations in PEX1 gene is associated with Heimler Syndrome. PMID: 26387595
7. results suggested that peroxisome biogenesis requires Pex1p- and Pex6p-regulated dissociation of Pex14p from Pex26p PMID: 25016021
8. the variants in PEX genes of a family PMID: 23247051
9. A 5' UTR polymorphism at position c.-53 and a promoter polymorphism 137 bp upstream of the PEX1 start codon are identified but strongly differing survival By genotype-phenotype analysis. PMID: 21846392
10. Missense mutations in PEX1 cause the milder forms of the peroxisome biogenesis disorders, whereas insertions, deletions, and nonsense mutations are associated with severe clinical phenotypes. PMID: 12032265
11. We have evaluated the impact of novel mutations, along with that of the two most common PEX1 mutations, in PBD patients by determining the levels of PEX1 mRNA, PEX1 protein, and peroxisome protein import. PMID: 12402331
12. complete lack of PEX1 is associated with Zellweger syndrome PMID: 12840548
13. overview of the currently known PEX1 mutations in Zellweger Syndrome [review] PMID: 16086329
14. analysis of PEX1 coding mutations and 5' UTR regulatory polymorphisms PMID: 16088892
15. Molecular confirmation of the clinical and biochemical diagnosis will allow the prediction of the clinical course of disease in individual PBD cases. PMID: 16141001
16. Insufficient binding to Pex1p x Pex6p complexes, or mislocalization of patient-derived Pex26p mutants is most likely responsible for the complementation group impaired peroxisome biogenesis. PMID: 16257970
17. Studies provide empirical data to estimate the relative fraction of disease-causing alleles that occur in the coding and splice junction sequences of PEX1 gene. PMID: 19105186
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相關疾?。?/div>
Peroxisome biogenesis disorder complementation group 1 (PBD-CG1); Peroxisome biogenesis disorder 1A (PBD1A); Peroxisome biogenesis disorder 1B (PBD1B); Heimler syndrome 1 (HMLR1)
亞細胞定位：

Cytoplasm. Peroxisome membrane. Note=Associated with peroxisomal membranes.
蛋白家族：

AAA ATPase family
數據庫鏈接：

HGNC: 8850

OMIM: 214100

KEGG: hsa:5189

STRING: 9606.ENSP00000248633

UniGene: Hs.164682

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