protein

HSD17B10 recombinant protein

MBS515351

0.02 mg

270 USD

Recombinant Protein

HSD17B10 recombinant protein

[HSD17B10]

[Hydroxysteroid (17-beta) Dehydrogenase 10; ABAD; ERAB; HCD2; MHBD; HADH2; SCHAD; 17b-HSD10; CAMR; MRPP2; MRX17; MRX31; MRXS10; SDR5C1; DUPXp11.22]

[Homo sapiens hydroxysteroid (17-beta) dehydrogenase 10 (HSD17B10), transcript variant 1, mRNA; 3-hydroxyacyl-CoA dehydrogenase type-2; 3-hydroxyacyl-CoA dehydrogenase type-2; mitochondrial RNase P subunit 2; AB-binding alcohol dehydrogenase; mitochondrial ribonuclease P protein 2; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase; short chain type dehydrogenase/reductase XH98G2; amyloid-beta peptide binding alcohol dehydrogenase; short chain L-3-hydroxyacyl-CoA dehydrogenase type 2; short chain dehydrogenase/reductase family 5C, member 1; endoplasmic reticulum-associated amyloid beta-peptide-binding protein; hydroxysteroid (17-beta) dehydrogenase 10; 17-beta-hydroxysteroid dehydrogenase 10 (EC:1.1.1.51); 17-beta-HSD 10; 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 type dehydrogenase/reductase XH98G2; Type II HADH]

[HSD17B10]

[ABAD; ERAB; HCD2; MHBD; HADH2; SCHAD; 17b-HSD10]

[HSD17B10; HSD17B10; ABAD; CAMR; ERAB; HCD2; MHBD; HADH2; MRPP2; MRX17; MRX31; SCHAD; MRXS10; SDR5C1; 17b-HSD10; DUPXp11.22; ERAB; HADH2; MRPP2; SCHAD; XH98G2; 17-beta-HSD 10; Mitochondrial RNase P protein 2]

HCD2_HUMAN

Sf9 insect cells

963

>95%

Recombinant protein stored in 50mM Tris-HCl, pH 7.5, 150mM NaCl, 10mM glutathione, 0.1mM EDTA, 0.25mM DTT, 0.1mM PMSF, 25% glycerol

0.2ug/ul

Storage :Store product at -70 degree C. For optimal storage, aliquot target into smaller quantities after centrifugation and store at recommended temperature. For most favorable performance, avoid repeated handling and multiple freeze/thaw cycles. Expiration : 1 year at -70 degree C from date of shipment. Shipping : Ships with dry ice.

Western Blot (WB)

SDS-PAGE

Type: Recombinant Fusion Protein. Species: Human. Tag Information: GST tag. Expression System: Sf9 insect cells using baculovirus. Source Note: Recombinant full-length human HSD17B10 was expressed using baculovirus in Sf9 cells

Cellular Proteins; Signaling Proteins - Cellular Proteins

Recombinant full-length human HSD17B10 was expressed by baculovirus in Sf9 insect cells using an N-terminal GST tag. The gene accession number is NM_004493. Scientific Backround :HSD17B10 gene encodes the protein 17-beta-hydroxysteroid dehydrogenase 10 that is a member of the short-chain dehydrogenase/reductase superfamily SCHAD (1). HSD17B10 gene product is a mitochondrial protein that is involved in lipid metabolism, fatty acid oxidation and steroid hormone metabolism. HSD17B10 protein has been implicated in the development of Alzheimer's disease and mutations in the gene are the cause of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD). Furthermore, HSD17B10 may act as a direct molecular link between beta-amyloid and mitochondrial toxicity (2).

1. Yang, S.-Y. et al: Multiple functions of type 10 17-beta-hydroxysteroid dehydrogenase. Trends Endocr. Metab. 16: 167-175, 2005. 2. Lustbader, J. W. et al: ABAD directly links A-beta to mitochondrial toxicity in Alzheimer's disease. Science 304: 448-452, 2004.

91823611

NM_004493

NM_004493

Q99714

~51 kDa

Alzheimer's Disease Pathway (83097); Alzheimer's Disease Pathway (509); Alzheimers Disease Pathway (672448); Branched-chain Amino Acid Catabolism Pathway (106179); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Tryptophan Metabolism Pathway (198850); Valine, Leucine And Isoleucine Degradation Pathway (82952); Valine, Leucine And Isoleucine Degradation Pathway (316); Fatty Acid Beta-oxidation I Pathway (142421)

This gene encodes 3-hydroxyacyl-CoA dehydrogenase type II, a member of the short-chain dehydrogenase/reductase superfamily. The gene product is a mitochondrial protein that catalyzes the oxidation of a wide variety of fatty acids, alcohols, and steroids. The protein has been implicated in the development of Alzheimer's disease, and mutations in the gene are the cause of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD). Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. [provided by RefSeq, Jul 2008]

Function: Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. Catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). Ref.1 Ref.10 Ref.13. Catalytic activity: (S)-3-hydroxyacyl-CoA + NAD+ = 3-oxoacyl-CoA + NADH. Ref.4 Ref.10(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD+ = 2-methylacetoacetyl-CoA + NADH. Ref.4 Ref.10Testosterone + NAD(P)+ = androst-4-ene-3,17-dione + NAD(P)H. Ref.4 Ref.10. Subunit structure: Homotetramer . By similarity. Interacts with MRPP1/TRMT10C and MRPP3/KIAA0391. Ref.13. Subcellular location: Mitochondrion Ref.10 Ref.13. Tissue specificity: Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD. Ref.1. Involvement in disease: 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD deficiency) [MIM:300438]: A disorder that leads to neurological abnormalities, including psychomotor retardation and, in virtually all patients, loss of mental and motor skills.Note: The disease is caused by mutations affecting the gene represented in this entry.Mental retardation, X-linked, syndromic, 10 (MRXS10) [MIM:300220]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. MRXS10 patients manifest mild mental retardation, choreoathetosis and abnormal behavior.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11Mental retardation, X-linked 17 (MRX17) [MIM:300705]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. Intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation, while syndromic mental retardation presents with associated physical, neurological and/or psychiatric manifestations.Note: The gene represented in this entry is involved in disease pathogenesis. A chromosomal microduplication involving HSD17B10 and HUWE1 has been found in patients with mental retardation. Ref.12. Sequence similarities: Belongs to the short-chain dehydrogenases/reductases (SDR) family. Biophysicochemical propertiesKinetic parameters:KM=25.7 uM for acetoacetyl-CoA (in the presence of 0.2 mM NADH, at pH 7.0 and 25 degrees Celsius) Ref.10KM=85.2 uM for beta-hydroxybutyryl-CoA (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=41 uM for androsterone (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=5 uM for 5-alpha-pregnan-20-beta-ol-3-one (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=219 uM for isoursodeoxycholic acid (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=36.4 uM for chenodeoxycholic acid (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=1.7 uM for dehydrocorticosterone (in the presence of 1 mM NAD, at pH 9.3 and 25 degrees Celsius)KM=30.6 uM for NADH (in the presence of acetoacetyl-CoA, at pH 7.0 and 25 degrees Celsius)KM=42.3 uM for NAD (in the presence of beta-hydroxybutyryl-CoA, at pH 9.3 and 25 degrees Celsius)pH dependence:Optimum pH is 9.3 for the dehydrogenase reaction at 25 degrees Celsius, and 7.0 for the reductase reaction at 25 degrees Celsius.

0.02 mg

270 USD

0.05 mg

450