HSD17B10 Antibody

Code CSB-PA002392
Size US$100
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  • Western Blot analysis of COS7 cells using ERAB Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
HSD17B10
Alternative Names
17 beta hydroxysteroid dehydrogenase 10 antibody; 17 beta hydroxysteroid dehydrogenase type 10 antibody; 17-beta-HSD 10 antibody; 17-beta-hydroxysteroid dehydrogenase 10 antibody; 17b HSD10 antibody; 3 hydroxy 2 methylbutyryl CoA dehydrogenase antibody; 3 hydroxyacyl CoA dehydrogenase type 2 antibody; 3 hydroxyacyl CoA dehydrogenase type II antibody; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase antibody; 3-hydroxyacyl-CoA dehydrogenase type II antibody; 3-hydroxyacyl-CoA dehydrogenase type-2 antibody; AB binding alcohol dehydrogenase antibody; ABAD antibody; Ads9 antibody; Amyloid beta binding polypeptide antibody; Amyloid beta peptide binding alcohol dehydrogenase antibody; Amyloid beta peptide binding protein antibody; Amyloid beta peptide binding protein antibody; CAMR antibody; DUPXp11.22 antibody; Endoplasmic Reticulum Amyloid Binding Protein antibody; Endoplasmic reticulum associated amyloid beta peptide binding protein antibody; Endoplasmic reticulum-associated amyloid beta-peptide-binding protein antibody; ER associated amyloid beta-binding protein antibody; ERAB antibody; HADH 2 antibody; HADH2 antibody; HCD 2 antibody; HCD2 antibody; HCD2_HUMAN antibody; Hsd17b10 antibody; Hydroxyacyl CoA Dehydrogenase type II antibody; Hydroxyacyl Coenzyme A dehydrogenase type II antibody; Hydroxysteroid (17 beta) dehydrogenase 10 antibody; Mental retardation X linked syndromic 11 antibody; MHBD antibody; Mitochondrial L3 Hydroxyacyl CoA Dehydrogenase antibody; Mitochondrial ribonuclease P protein 2 antibody; Mitochondrial RNase P protein 2 antibody; MRPP2 antibody; MRX17 antibody; SCHAD antibody; SDR5C1 antibody; Short chain dehydrogenase/reductase family 5C member 1 antibody; Short chain L 3 hydroxyacyl CoA dehydrogenase type 2 antibody; Short chain type dehydrogenase/reductase XH98G2 antibody; Short-chain type dehydrogenase/reductase XH98G2 antibody; Type 10 17b HSD antibody; Type 10 17beta hydroxysteroid dehydrogenase antibody; Type II HADH antibody; XH98G2 antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat,Monkey
Immunogen
Synthesized peptide derived from the Internal region of Human ERAB.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
ELISA 1:40000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.

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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease
HDS10 mitochondrial disease (HSD10MD); Mental retardation, X-linked 17 (MRX17)
Subcellular Location
Mitochondrion. Mitochondrion matrix, mitochondrion nucleoid.
Protein Families
Short-chain dehydrogenases/reductases (SDR) family
Tissue Specificity
Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD.
Database Links

HGNC: 4800

OMIM: 300256

KEGG: hsa:3028

STRING: 9606.ENSP00000168216

UniGene: Hs.171280

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