ECHS1 Antibody

說明書

中文名稱：

ECHS1兔多克隆抗體
貨號：

CSB-PA007382GA01HU
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.：

P30084
基因名：

ECHS1
別名：

ECHS1Enoyl-CoA hydratase antibody; mitochondrial antibody; EC 4.2.1.17 antibody; Enoyl-CoA hydratase 1 antibody; Short-chain enoyl-CoA hydratase antibody; SCEH antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human ECHS1
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：

Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate.
基因功能參考文獻：
1. Due to enoyl coenzyme A hydratase, short chain, 1, mitochondria (ECHS1) deficiency. PMID: 28409271
2. exceeding nutrients suppress Enoyl-CoA hydratase-1 (ECHS1) activity by inducing its acetylation resulting in accumulation of fatty acids and branched-chain amino acids and oncogenic mTOR activation PMID: 28878358
3. ECHS1 mutations phenotype might be milder than reported earlier, compatible with prolonged survival, and also includes isolated paroxysmal exercise-induced dystonia. PMID: 27090768
4. Whole exome sequencing identified heterozygous ECHS1 mutations c.836T>C (novel) and c.8C>A for short-chain enoyl-CoA hydratase (SCEH) deficiency of which 1/2 of the cases are associated with secondary lymphocyte pyruvate dehydrogenase complex deficiency.[review] PMID: 28202214
5. This is the first report of homozygosity for a truncating mutation in ECHS1, which may explain the severe phenotype. Our report highlights the need to consider SCEH deficiency in patients with lethal neonatal lactic acidosis PMID: 27905109
6. Human ECHS1 catalyses the hydration of five substrates via different metabolic pathways, with the highest specificity for crotonyl-CoA and the lowest specificity for tiglyl-CoA PMID: 26251176
7. These results suggested that ECHS1 may promote cell proliferation in hepatocellular carcinoma in an EGFR-dependent manner. PMID: 25760819
8. In conclusion, the results of the present study suggested that ECHS1 may have an important role in colorectal cancer cell proliferation and migration PMID: 25739098
9. identification of four additional patients with mutations in ECHS1 highlights the importance of the valine degradation pathway in Leigh syndrome PMID: 26099313
10. ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome. PMID: 25393721
11. ECHS1 may play important roles in gastric cancer cell proliferation and migration through PKB- and GSK3b-related signaling pathways. PMID: 25338767
12. The study demonstrated that ECHS1 mutations result in ECHS1 deficiency and are another cause of Leigh disease in two siblings. PMID: 25125611
13. ECHS1 knockdown reduced cell viability and enhanced cisplatin-induced apoptosis in hepatocellular carcinoma cells. PMID: 23879543
14. The results confirmed that small hepatitis B surface antigen (SHBs) interacted with ECHS1. PMID: 23275097
15. The coexistence of HBs and ECHS1 enhances HepG2 cell apoptosis, affects ECHS1 localization in the mitochondria and induces apoptosis by decreasing the mitochondrial membrane potential (MMP). PMID: 23178449
16. ECHS1 specifically represses STAT3 activity and negatively regulates the expression of several target genes of STAT3 through inhibiting STAT3 phosphorylation. PMID: 23416296
17. Expression of mitochondrial short chain enoyl-CoA hydratase (ECHS)was significantly down-modulated in virus infected glioblastoma cells and ECHS knockdown (siRNA) impaired virus replication and cytopathic effects. PMID: 17395278
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相關疾病：

Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (ECHS1D)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Enoyl-CoA hydratase/isomerase family
組織特異性：

Liver, fibroblast, muscle. Barely detectable in spleen and kidney.
數據庫鏈接：

HGNC: 3151

OMIM: 602292

KEGG: hsa:1892

STRING: 9606.ENSP00000357535

UniGene: Hs.76394

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