PDHX Antibody

說明書

中文名稱：

PDHX兔多克隆抗體
貨號：

CSB-PA017718GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

O00330
基因名：

PDHX
別名：

Dihydrolipoamide dehydrogenase binding protein of pyruvate dehydrogenase complex antibody; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex antibody; DLDBP antibody; E3 binding protein antibody; E3-binding protein antibody; E3BP antibody; Lipoyl containing pyruvate dehydrogenase complex component X antibody; Lipoyl-containing pyruvate dehydrogenase complex component X antibody; mitochondrial antibody; ODPX_HUMAN antibody; OPDX antibody; PDHX antibody; PDX 1 antibody; PDX1 antibody; Pro X antibody; proX antibody; Pyruvate dehydrogenase complex component X antibody; Pyruvate dehydrogenase complex lipoyl containing component X antibody; Pyruvate dehydrogenase complex; E3 binding protein subunit antibody; Pyruvate dehydrogenase protein X component antibody; Pyruvate dehydrogenase protein X component mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human PDHX
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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) 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.

產品評價

靶點詳情

功能：

Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
基因功能參考文獻：
1. We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis. PMID: 25087164
2. MiR-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX. PMID: 24935220
3. New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase deficiency. PMID: 22766002
4. genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 was established. PMID: 21194677
5. determination that PDH and complex III exist at a steady-state ratio of 1:100, 1:128 and 1:202 in HeLa cell extracts, fibroblast mitochondria and heart tissue mitochondria, respectively PMID: 12372595
6. model of the pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and binding of the E1 and E3 components PMID: 14638692
7. specificity of pairing for human E3BP with E3 from its subcomplex structure to be most likely due to conformational rigidity of the binding fragment of the E3-binding domain of E3BP and its exquisite amino acid match with the E3 target interface PMID: 16263718
8. A cluster of disease-causing E3 mutations located near the center of the E3BD/E3 interface prevents the efficient recruitment of these E3 variants by E3BP into the PDC, leading to the dysfunction of the PDC catalytic machine. PMID: 16442803
9. These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene. PMID: 16566017
10. Despite the presence of antibodies reactive with PDC-E3BP in the majority of primary biliary cirrhosis (PBC) patients this self-protein is not a dominant T-cell autoantigen in PBC. PMID: 16629643
11. PDHX-assisted photosensitization with rose Bengal induces structural and functional alteration of mitochondria in HeLa cells. PMID: 17024456
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相關疾病：

Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

2-oxoacid dehydrogenase family
數據庫鏈接：

HGNC: 21350

OMIM: 245349

KEGG: hsa:8050

STRING: 9606.ENSP00000227868

UniGene: Hs.502315

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