ABHD5 Recombinant Monoclonal Antibody

說明書

中文名稱：

ABHD5 Recombinant Monoclonal Antibody
貨號：

CSB-RA982153A0HU
規格：

￥1320
圖片：
Flow cytometric analysis of ABHD5 expression in C2C12 cells using ABHD5 antibody. Green, isotype control; red, ABHD5.

Immunocytochemical staining of C2C12 cells with ABHD5 antibody. Nuclei were stained blue with DAPI; ABHD5 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Low. Scale bar, 20 μm.

Western blotting analysis using ABHD5 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with ABHD5 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q8WTS1
基因名：

ABHD5
別名：

ABHD5; Antibodyhydrolase Domain Containing 5, Lysophosphatidic Acid Acyltransferase; NCIE2; 1-Acylglycerol-3-Phosphate O-Acyltransferase ABHD5; Antibodyhydrolase Domain-Containing Protein 5; Lipid Droplet-Binding Protein CGI-58; EC 2.3.1.51; CGI-58; Antibodyhydrolase Domain Containing 5; CGI58; IECN2
反應種屬：

Human, Mouse, Rat
免疫原：

Recombinant Human ABHD5 protein
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

3G6
濃度：

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

Rabbit IgG in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, FC, ICC

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
FC	1:200-1:2000
ICC	1:100-1:1000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Flow cytometry (FC) 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：

Coenzyme A-dependent lysophosphatidic acid acyltransferase that catalyzes the transfert of an acyl group on a lysophosphatidic acid. Functions preferentially with 1-oleoyl-lysophosphatidic acid followed by 1-palmitoyl-lysophosphatidic acid, 1-stearoyl-lysophosphatidic acid and 1-arachidonoyl-lysophosphatidic acid as lipid acceptor. Functions preferentially with arachidonoyl-CoA followed by oleoyl-CoA as acyl group donors. Functions in phosphatidic acid biosynthesis. May regulate the cellular storage of triacylglycerol through activation of the phospholipase PNPLA2. Involved in keratinocyte differentiation. Regulates lipid droplet fusion.
基因功能參考文獻：
1. our findings indicate that inhibition of both DGAT1 and ABHD5 using siRNA leads to reduction in prostate cancer cell growth. PMID: 28877685
2. Prebinding CGI-58 with PI(3)P or PI(5)P did not alter its coactivation of ATGL in vitro. In summary, purified recombinant CGI-58 that is functional as an ATGL coactivator lacks LPAAT activity. PMID: 24879803
3. It is clear that CGI-58 can regulate TAG hydrolysis by activating the major TAG hydrolase adipose triglyceride lipase (ATGL), yet CGI-58 can also regulate lipid metabolism via mechanisms that do not involve ATGL. PMID: 28827091
4. ABHD5 possesses a PNPLA2-independent function in regulating autophagy and tumorigenesis. PMID: 27559856
5. These results indicate that HCV taps into the lipid droplet triglyceride reservoir usurping ABHD5 lipase cofactor function PMID: 27124600
6. Case Report: novel ABHD5 mutation, c.838C > T (p.Arg280*), in trans with p.Arg234* in a Chinese patient with very mild Dorfman-Chanarin syndrome. PMID: 26547112
7. Authors show that rat ATGL, coactivated by rat CGI-58, efficiently hydrolyzes triglycerides and retinyl ester. PMID: 26330055
8. novel ABHD5 truncating variant in a twenty nine month old female child, who presented with icthyosiform erythroderma. PMID: 26353074
9. simultaneous tryptophan alanine permutations in both arms abolish localization and activity of CGI-58 as opposed to tryptophan substitutions that occur in only one arm. PMID: 26350461
10. this study presents clinical and molecular data of four affected relatives with Chanarin-Dorfman syndrome homozygous for a N209X mutation in ABHD5, and provides a short review by comparing patients with N209X homozygous mutations to patients with other ABHD5 mutations. PMID: 25682902
11. PLIN5 was significantly colocated with ATGL, mitochondria and CGI-58, indicating a close association between the key lipolytic effectors in resting skeletal muscle. PMID: 25054327
12. -mediated phosphorylation of CGI-58 is required for dispersion of CGI-58 from perilipin 1A-coated lipid droplets PMID: 25421061
13. Abhd5 expression falls substantially and correlates negatively with malignant features in human colorectal cancer. PMID: 25482557
14. Findings indicate a molecular mechanisms by which lysophosphatidic acid acyltransferase CGI-58 regulates lipid homeostasis. PMID: 25315780
15. A report of two Chanarin-Dorfman syndrome sisters with severe steatohepatitis and decompensated cirrhosis due to steatohepatitis in whom the clinical presentation developed due to a new mutation in ABHD5 gene. PMID: 22245374
16. an important metabolic function of CGI-58 in skeletal muscle PMID: 22383684
17. A novel nonsense mutation of ABHD5 is reported in a consanguineous Afgani family with 4 sibs with Dorfman Chanarin syndrome; the mutation leads to protein truncation by 14 AAs; findings include liver cirrhosis, corneal opacities,tessellated fundus PMID: 22373837
18. the C terminus sequesters ABHD5 and thus inhibits basal ATGL activity PMID: 21757733
19. First report of large genomic deletions in the ABHD5 gene in Chanarin-Dorfman syndrome patients from Mediterranean countries. PMID: 21122093
20. analysis of novel mutations in the ABHD5 gene in Chanarin-Dorfman syndrome (CDS) patients PMID: 20307695
21. CGI-58 not only facilitates triglyceride lipolysis, but also provides fatty acid for esterification of ceremide leading to acylceremides PMID: 20520629
22. ABDH5 gene mutation is found in patients diagnosed with Chanarin Dorfman syndrome. PMID: 15967942
23. CGI-58 interacts with adipose triglyceride lipase, stimulating its TG hydrolase activity up to 20-fold PMID: 16679289
24. CGI-58 facilitates lipolysis in cooperation with perilipin and other factors, including lipases PMID: 17308334
25. analysis of a novel S115G mutation of CGI-58 in a Turkish patient with Dorfman-Chanarin syndrome [case report] PMID: 17495960
26. Deficient in lipid storage diseases. PMID: 17631826
27. These data enriches the list of CGI-58 mutations associated with Chanarin-Dorfman syndrome and confirms the clinical and allelic heterogeneity of this disease, especially the inconsistent muscle involvement. PMID: 18339307
28. CGI-58, the causative gene for Chanarin-Dorfman syndrome, mediates acylation of lysophosphatidic acid PMID: 18606822
29. CGI-58 is expressed and packaged into lamellar granules (LG) during keratinization and likely plays crucial role(s) in keratinocyte differentiation and LG lipid metabolism, contributing to skin lipid barrier formation. PMID: 18832586
30. Chanarin-Dorfman syndrome: deficiency in CGI-58, a lipid droplet-bound coactivator of lipase. (Review) PMID: 19061969
31. review summarizes recent findings with the goal of relating structural variants of ATGL and CGI-58 to functional consequences in lipid metabolism [review] PMID: 19401457
32. mutational analysis in Chanarin-Dorfman syndrome PMID: 11590543
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相關疾病：

Chanarin-Dorfman syndrome (CDS)
亞細胞定位：

Cytoplasm. Lipid droplet. Cytoplasm, cytosol.
蛋白家族：

Peptidase S33 family, ABHD4/ABHD5 subfamily
組織特異性：

Widely expressed in various tissues, including lymphocytes, liver, skeletal muscle and brain. Expressed by upper epidermal layers and dermal fibroblasts in skin, hepatocytes and neurons (at protein level).
數據庫鏈接：

HGNC: 21396

OMIM: 275630

KEGG: hsa:51099

STRING: 9606.ENSP00000390849

UniGene: Hs.19385

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