HSD17B10 Antibody

說明書

中文名稱：

HSD17B10兔多克隆抗體
貨號：

CSB-PA695225
規格：

￥2024
圖片：
Western blot analysis of extract from JK, HUVEC, HepG2 and Hela cells using ERAB Antibody
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) HSD17B10 Polyclonal antibody
Uniprot No.：

Q99714
基因名：

HSD17B10
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Peptide sequence around aa.253~257(L-D-G-A-I)derived from Human ERAB.
免疫原種屬：

Homo sapiens (Human)
克隆類型：

Polyclonal
純化方式：

Antibodies were produced by immunizing rabbits with synthetic peptide and KLH conjugates. Antibodies were purified by affinity-chromatography using epitope-specific peptide.
濃度：

It differs from different batches. Please contact us to confirm it.
產品提供形式：

Liquid
應用范圍：

ELISA,WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:1000

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.

產品評價

靶點詳情

功能：

Mitochondrial dehydrogenase involved in pathways of fatty acid, branched-chain amino acid and steroid metabolism. Acts as (S)-3-hydroxyacyl-CoA dehydrogenase in mitochondrial fatty acid beta-oxidation, a major degradation pathway of fatty acids. Catalyzes the third step in the beta-oxidation cycle, namely the reversible conversion of (S)-3-hydroxyacyl-CoA to 3-ketoacyl-CoA. Preferentially accepts straight medium- and short-chain acyl-CoA substrates with highest efficiency for (3S)-hydroxybutanoyl-CoA. Acts as 3-hydroxy-2-methylbutyryl-CoA dehydrogenase in branched-chain amino acid catabolic pathway. Catalyzes the oxidation of 3-hydroxy-2-methylbutanoyl-CoA into 2-methyl-3-oxobutanoyl-CoA, a step in isoleucine degradation pathway. Has hydroxysteroid dehydrogenase activity toward steroid hormones and bile acids. Catalyzes the oxidation of 3alpha-, 17beta-, 20beta- and 21-hydroxysteroids and 7alpha- and 7beta-hydroxy bile acids. Oxidizes allopregnanolone/brexanolone at the 3alpha-hydroxyl group, which is known to be critical for the activation of gamma-aminobutyric acid receptors (GABAARs) chloride channel. Has phospholipase C-like activity toward cardiolipin and its oxidized species. Likely oxidizes the 2'-hydroxyl in the head group of cardiolipin to form a ketone intermediate that undergoes nucleophilic attack by water and fragments into diacylglycerol, dihydroxyacetone and orthophosphate. Has higher affinity for cardiolipin with oxidized fatty acids and may degrade these species during the oxidative stress response to protect cells from apoptosis. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). Essential for structural and functional integrity of mitochondria.; In addition to mitochondrial dehydrogenase activity, moonlights as a component of mitochondrial ribonuclease P, a complex that cleaves tRNA molecules in their 5'-ends. Together with TRMT10C/MRPP1, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity. The MRPP1-MRPP2 subcomplex catalyzes the formation of N(1)-methylguanine and N(1)-methyladenine at position 9 (m1G9 and m1A9, respectively) in tRNAs; HSD17B10/MRPP2 acting as a non-catalytic subunit. The MRPP1-MRPP2 subcomplex also acts as a tRNA maturation platform: following 5'-end cleavage by the mitochondrial ribonuclease P complex, the MRPP1-MRPP2 subcomplex enhances the efficiency of 3'-processing catalyzed by ELAC2, retains the tRNA product after ELAC2 processing and presents the nascent tRNA to the mitochondrial CCA tRNA nucleotidyltransferase TRNT1 enzyme. Associates with mitochondrial DNA complexes at the nucleoids to initiate RNA processing and ribosome assembly.
基因功能參考文獻：
1. Authors report two patients with novel missense mutations in the HSD17B10 gene (c.34G>C and c.526G>A), resulting in the p.V12L and p.V176M substitutions. Val12 and Val176 are highly conserved residues located at different regions of the MRPP2 structure. PMID: 28888424
2. in addition to being an essential component of the RNase P reaction, MRPP1/2 serves as a processing platform for several down-stream tRNA maturation steps in human mitochondria. PMID: 29040705
3. The S-nitrosation of a cysteine residue distal to the 3-hydroxyacyl-CoA dehydrogenase type 2 (HADH2) active site impaired catalytic activity. PMID: 27291402
4. A computational study and enzyme inhibition assay with full length human 17-beta-HSD10 identifies risperidone as enzyme inhibitor and possible antineoplastic agent. PMID: 28188816
5. Data suggest that HSD10 plays a role in alterations of energy metabolism by regulating mtDNA content in colorectal carcinomas. PMID: 26884257
6. Our findings demonstrate that overexpression of HSD10 accelerates pheochromocytoma cell growth, enhances cell respiration, and increases cellular resistance to cell death induction. PMID: 25879199
7. Three HSD10 variants associated with neurodegenerative disorders are inactive with cardiolipin PMID: 26338420
8. The authors demonstrate elevated amounts of unprocessed pre-tRNAs and mRNA transcripts encoding mitochondrial subunits indicating deficient RNase P activity in HSD10 disease. PMID: 25575635
9. The study showed that pathogenic mutations impair SDR5C1-dependent dehydrogenation, tRNA processing and methylation. PMID: 25925575
10. loss of HSD10 causes impaired mitochondrial precursor transcript processing which may explain mitochondrial dysfunction observed in HSD10 disease PMID: 24549042
11. Defects in this gene are a cause of 17beta-hydroxysteroid dehydrogenase type 10 (HSD10) deficiency. The encoded protein does not exhibit generalized alcohol dehydrogenase activity as was previously thought. PMID: 25007702
12. Significantly higher levels of SRD5A1, AKR1C2, AKR1C3, and HSD17B10 mRNA were however found in bone metastases than in non-malignant and/or malignant prostate tissue PMID: 24244276
13. Inhibition of mitochondrial RNase P by beta-amyloid is an unspecific effect and is not mediated by beta-amyloid interaction with SDR5C1. PMID: 23755257
14. Two major HSD17B10 transcription start sites were identified by primer extension at -37 and -6 as well as a minor start site at -12 nucleotides from the initiation codon ATG. PMID: 23834306
15. A 5-methylcytosine is present in both active and inactive X chromosomes at + 2259 nucleotide from the initiation ATG of the HSD17B10 gene, explaining the prevalence of the p.R130C mutation among HSD10 deficiency patients. PMID: 23266819
16. analysis of clinical consequences of mutations in the HSD17B10 gene PMID: 22127393
17. The role of ABAD in amyloid beta toxicity, was investigated. PMID: 22174920
18. behavioral stress causes protein up-regulation in the brain of a mouse model of Alzheimer disease PMID: 21382475
19. These results suggest that the HSD17B10 gene does not escape X-inactivation as has been reported previously. PMID: 20664630
20. HSD17B10 is regulated by several isoforms of C/EBP-beta in HepG2 cells. PMID: 20638476
21. This finding indicates that the symptoms in patients with mutations in the HSD17B10 gene are unrelated to accumulation of toxic metabolites in the isoleucine pathway and, rather, related to defects in general mitochondrial function. PMID: 20077426
22. Sequence analysis of the HADH2 gene from patients with MHBD deficiency revealed the presence of two missense mutations (R130C and L122V)which almost completely abolish enzyme activity PMID: 12696021
23. Comparison of substrate specificity of human and Drosophila melanogaster type 10 17b-hydroxysteroid dehydrogenases PMID: 12917011
24. Abeta interacts with ABAD in the mitochondria of Alzheimer's disease patients and transgenic mice; data suggest that the ABAD-Abeta interaction may be a therapeutic target in Alzheimer's disease PMID: 15087549
25. crystal structure of ABAD/HSD10 complexed with NAD(+) and an inhibitory small molecule PMID: 15342248
26. findings link amyloid-beta peptide (Abeta) binding alcohol dehydrogenase (ABAD)-induced oxidant stress to critical aspects of Alzheimer's disease (AD)-associated cellular dysfunction, suggesting a pivotal role for this enzyme in the pathogenesis of AD PMID: 15665036
27. Brain astrocytes contain a moderate level of 17beta-HSD10, which is elevated in activated astrocytes of brains with Alzheimer type pathology, including sporadic Alzheimer's disease (AD) and Down syndrome with AD. PMID: 15804423
28. Reduced expression of the HADH2 protein causes MRXS10, a phenotype different from that caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency, which is a neurodegenerative disorder caused by missense mutations in this multifunctional protein. PMID: 17236142
29. These results propose an additional role of ABAD in neural cell death in AD. PMID: 17707551
30. Data suggest that thioredoxin could not only assist ABAD-inhibiting peptide expression, but rebalance the disturbed "redox equilibrium" caused by intracellular amyloid beta in PC12 cells. PMID: 17917077
31. Increased gene dosage of HSD17B10, HUWE1, or both contribute to the etiology of X-Linked Mental Retardation. PMID: 18252223
32. In Alzheimer disease and schizophrenia, significant shifts to left/right asymmetry were found and the changes were associated with more marked increases in mRNA/enzyme expression in the left hemisphere PMID: 18765932
33. Up-rulation of HSD17B10 expression is associated with poor response to chemotherapy in conventional osteosarcomas. PMID: 19449377
34. Amyloid-beta-peptide binding to mitochondrial Abeta-binding alcohol dehydrogenase (ABAD) enzyme triggers a series of events leading to mitochondrial dysfunction characteristic of Alzheimer's disease. PMID: 19601895
35. results support the theory that an imbalance in neurosteroid metabolism could be a major cause of the neurological handicap associated with hydroxysteroid (17beta) dehydrogenase 10 deficiency. PMID: 19706438
36. The data indicated pronounced increases in the 17beta-hydroxysteroid dehydrogenase type 10 levels, specifically to 179% in multiple sclerosis and to 573% in Alzheimer disease when compared to the age-matched controls. PMID: 19756307
37. Here, we demonstrate that Abeta-binding alcohol dehydrogenase (ABAD) is a direct molecular link from Abeta to mitochondrial toxicity. PMID: 15087549
38. tissue distribution, subcellular localization, and metabolic functions PMID: 11559359
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相關疾病：

HDS10 mitochondrial disease (HSD10MD); Mental retardation, X-linked 17 (MRX17)
亞細胞定位：

Mitochondrion. Mitochondrion matrix, mitochondrion nucleoid.
蛋白家族：

Short-chain dehydrogenases/reductases (SDR) family
組織特異性：

Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD.
數據庫鏈接：

HGNC: 4800

OMIM: 300256

KEGG: hsa:3028

STRING: 9606.ENSP00000168216

UniGene: Hs.171280

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