Alternative Name(s):

17-beta-hydroxysteroid dehydrogenase 10 (EC:1.1.1.51) ;17-beta-HSD 103-hydroxy-2-methylbutyryl-CoA dehydrogenase (EC:1.1.1.178)3-hydroxyacyl-CoA dehydrogenase type IIEndoplasmic reticulum-associated amyloid beta-peptide-binding protein;Mitochondrial ribonuclease P protein 2 ;Mitochondrial RNase P protein 2Short chain dehydrogenase/reductase family 5C member 1Short-chain type dehydrogenase/reductase XH98G2Type II HADH

Species: (Organism)

Homo sapiens (Human)

Gene Names:

HSD17B10

Tag info:

N-terminal 6xHis-SUMO-tagged

Target Protein AA Sequence:

AAACRSVKGLVAVITGGASGLGLATAERLVGQGASAVLLDLPNSGGEAQAKKLGNNCVFAPADVTSEKDVQTALALAKGKFGRVDVAVNCAGIAVASKTYNLKKGQTHTLEDFQRVLDVNLMGTFNVIRLVAGEMGQNEPDQGGQRGVIINTASVAAFEGQVGQAAYSASKGGIVGMTLPIARDLAPIGIRVMTIAPGLFGTPLLTSLPEKVCNFLASQVPFPSRLGDPAEYAHLVQAIIENPFLNGEVIRLDGAIRMQP

Expression Region:

2-261aa

Subcellular Location:

Mitochondrion

Tissue Specificity:

Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD.

Protein Length:

Full Length of Mature Protein

Pathway:

Mol. Weight:

42.8 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Transcription

Function:

Mitochondrial dehydrogenase that catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone

Involvement in disease:

HDS10 mitochondrial disease (HSD10MD); Mental retardation, X-linked 17 (MRX17)

Relevance:

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).

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Short-chain dehydrogenases/reductases (SDR) family

Reference:

An intracellular protein that binds amyloid-beta peptide and mediates neurotoxicity in Alzheimer's disease.Yan S.D., Fu J., Soto C., Chen X., Zhu H., Al-Mohanna F., Collinson K., Zhu A., Stern E., Saido T., Tohyama M., Ogawa S., Roher A., Stern D.Nature 389:689-695(1997)