Product Details

Uniprot No.

Target Names

HSD17B10

Alternative Names

3-hydroxyacyl-CoA dehydrogenase type-2 (EC 1.1.1.35) (17-beta-hydroxysteroid dehydrogenase 10) (17-beta-HSD 10) (EC 1.1.1.51) (2-methyl-3-hydroxybutyryl-CoA dehydrogenase) (MHBD) (3-hydroxy-2-methylbutyryl-CoA dehydrogenase) (EC 1.1.1.178) (3-hydroxyacyl-CoA dehydrogenase type II) (Endoplasmic reticulum-associated amyloid beta-peptide-binding protein) (Mitochondrial ribonuclease P protein 2) (Mitochondrial RNase P protein 2) (Short chain dehydrogenase/reductase family 5C member 1) (Short-chain type dehydrogenase/reductase XH98G2) (Type II HADH), HSD17B10, ERAB HADH2 MRPP2 SCHAD SDR5C1 XH98G2

Species Reactivity

Human, Mouse, Rat

Immunogen

A synthesized peptide from human HSD17B10 protein

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

12E12

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid

Tested Applications

ELISA, WB, IHC, FC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:50-1:200
FC	1:50-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody 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.

Description

HSD17B10 is a multifunctional mitochondrial enzyme with remarkable biological significance, participating in steroid hormone metabolism, fatty acid oxidation, and isoleucine catabolism. Beyond these metabolic roles, this enzyme serves as an essential component of mitochondrial RNase P and has gained considerable attention for its interaction with amyloid beta-peptide, positioning it as a key target in neurodegeneration research, metabolic disorder studies, and cancer biology investigations.

This recombinant monoclonal antibody, generated against a synthetic peptide derived from human HSD17B10, offers the reproducibility and consistency that demanding research protocols require. As a sequence-defined reagent produced through recombinant technology, it eliminates the lot-to-lot variability that can compromise longitudinal studies and ensures reliable performance across your experimental timeline.

Extensive validation demonstrates this antibody's versatility across multiple applications. Western blot analysis confirms specific detection at the expected 27 kDa molecular weight across diverse human cell lines including HeLa, MCF-7, Colo205, A549, HEK293, SH-SY5Y, and THP-1 cells, with recommended dilutions ranging from 1:500 to 1:2000. Cross-species reactivity extends to mouse and rat samples, validated through detection in brain tissue lysates from both species, supporting translational research using rodent models. Immunohistochemistry applications have been validated in human liver cancer and thyroid tissue sections at 1:100 dilution, while flow cytometry analysis demonstrates clear positive staining in MCF-7 cells, enabling quantitative single-cell studies.

Whether investigating mitochondrial dysfunction in neurodegenerative disease models, exploring metabolic reprogramming in cancer, or characterizing steroid hormone pathways, this antibody provides a dependable tool for advancing your research across protein expression, tissue localization, and cellular analysis workflows.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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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

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
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
The S-nitrosation of a cysteine residue distal to the 3-hydroxyacyl-CoA dehydrogenase type 2 (HADH2) active site impaired catalytic activity. PMID: 27291402
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
Data suggest that HSD10 plays a role in alterations of energy metabolism by regulating mtDNA content in colorectal carcinomas. PMID: 26884257
Our findings demonstrate that overexpression of HSD10 accelerates pheochromocytoma cell growth, enhances cell respiration, and increases cellular resistance to cell death induction. PMID: 25879199
Three HSD10 variants associated with neurodegenerative disorders are inactive with cardiolipin PMID: 26338420
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
The study showed that pathogenic mutations impair SDR5C1-dependent dehydrogenation, tRNA processing and methylation. PMID: 25925575
loss of HSD10 causes impaired mitochondrial precursor transcript processing which may explain mitochondrial dysfunction observed in HSD10 disease PMID: 24549042
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
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
Inhibition of mitochondrial RNase P by beta-amyloid is an unspecific effect and is not mediated by beta-amyloid interaction with SDR5C1. PMID: 23755257
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
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
analysis of clinical consequences of mutations in the HSD17B10 gene PMID: 22127393
The role of ABAD in amyloid beta toxicity, was investigated. PMID: 22174920
behavioral stress causes protein up-regulation in the brain of a mouse model of Alzheimer disease PMID: 21382475
These results suggest that the HSD17B10 gene does not escape X-inactivation as has been reported previously. PMID: 20664630
HSD17B10 is regulated by several isoforms of C/EBP-beta in HepG2 cells. PMID: 20638476
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
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
Comparison of substrate specificity of human and Drosophila melanogaster type 10 17b-hydroxysteroid dehydrogenases PMID: 12917011
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
crystal structure of ABAD/HSD10 complexed with NAD(+) and an inhibitory small molecule PMID: 15342248
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
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
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
These results propose an additional role of ABAD in neural cell death in AD. PMID: 17707551
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
Increased gene dosage of HSD17B10, HUWE1, or both contribute to the etiology of X-Linked Mental Retardation. PMID: 18252223
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
Up-rulation of HSD17B10 expression is associated with poor response to chemotherapy in conventional osteosarcomas. PMID: 19449377
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
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
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
Here, we demonstrate that Abeta-binding alcohol dehydrogenase (ABAD) is a direct molecular link from Abeta to mitochondrial toxicity. PMID: 15087549
tissue distribution, subcellular localization, and metabolic functions PMID: 11559359

Hide All

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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Code	CSB-RA083975A0HU
Size	US$210
	Order now
Image	Western Blot Positive WB detected in: HeLa whole cell lysate(30µg), MCF-7 whole cell lysate(30µg), colo205 whole cell lysate(30µg), A549 whole cell lysate(30µg), HEK293 whole cell lysate(30µg), SH-SY5Y whole cell lysate(30µg), THP-1 whole cell lysate(30µg), Mouse brain tissue lysate(30µg), Rat brain tissue lysate(30µg) All lanes: HSD17B10 antibody at 1:1000 Secondary Goat polyclonal to rabbit IgG at 1/40000 dilution Predicted band size: 27 kDa Observed band size: 27 kDa Exposure time：1min IHC image of CSB-RA083975A0HU diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. IHC image of CSB-RA083975A0HU diluted at 1:100 and staining in paraffin-embedded human thyroid tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Overlay Peak curve showing MCF-7 cells stained with CSB-RA083975A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/110⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/110⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
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HSD17B10 Recombinant Monoclonal Antibody

Product Details

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Target Background