Product Name
HADHA, siRNA
Full Product Name
HADHA siRNA (Rat)
Product Synonym Names
Trifunctional enzyme subunit alpha mitochondrial; TP-alpha
Product Gene Name
HADHA sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q64428
Specificity
HADHA siRNA (Rat) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Purity/Purification
> 97%
Form/Format
Lyophilized powder
Quality Control
Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
Directions for Use
We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
Components
We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of rat HADHA gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of HADHA sirna vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
HADHA sirna
siRNA to inhibit HADHA expression using RNA interference
Applications Tested/Suitable for HADHA sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for HADHA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_570839.2
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NCBI GenBank Nucleotide #
NM_130826.2
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UniProt Primary Accession #
Q64428
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UniProt Secondary Accession #
Q5BIZ5[Other Products]
UniProt Related Accession #
Q64428[Other Products]
Molecular Weight
82,665 Da
NCBI Official Full Name
trifunctional enzyme subunit alpha, mitochondrial
NCBI Official Synonym Full Names
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
NCBI Official Symbol
Hadha [Similar Products]
NCBI Official Synonym Symbols
RGD1560655
[Similar Products]
NCBI Protein Information
trifunctional enzyme subunit alpha, mitochondrial
UniProt Protein Name
Trifunctional enzyme subunit alpha, mitochondrial
UniProt Synonym Protein Names
TP-alphaIncluding the following 2 domains:Long-chain enoyl-CoA hydratase (EC:4.2.1.17)Long chain 3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.211)
Protein Family
Trifunctional enzyme
UniProt Gene Name
Hadha [Similar Products]
UniProt Entry Name
ECHA_RAT
NCBI Summary for HADHA
alpha subunit of the liver mitochondrial fatty acid oxidation multienzyme complex [RGD, Feb 2006]
UniProt Comments for HADHA
HADHA: Bifunctional subunit. Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency). The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all enzyme activities of the TFP complex. Defects in HADHA are the cause of long-chain 3-hydroxyl- CoA dehydrogenase deficiency (LCHAD deficiency). The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long- chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced. Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP). AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).
Protein type: EC 1.1.1.211; Lyase; Acetyltransferase; Oxidoreductase; Amino Acid Metabolism - tryptophan; Lipid Metabolism - fatty acid elongation in mitochondria; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - butanoate; Other Amino Acids Metabolism - beta-alanine; Secondary Metabolites Metabolism - limonene and pinene degradation; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; Lipid Metabolism - unsaturated fatty acid biosynthesis; Mitochondrial; EC 4.2.1.17; Amino Acid Metabolism - lysine degradation
Cellular Component: mitochondrion; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex
Molecular Function: acyl-CoA binding; enoyl-CoA hydratase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; NAD binding; acetyl-CoA C-acyltransferase activity; 3-hydroxyacyl-CoA dehydrogenase activity
Biological Process: response to drug; fatty acid beta-oxidation; response to insulin stimulus
Research Articles on HADHA
1. The catalytic residues of the MTP beta-subunit are positioned in the active site similarly to those of monofunctional 3-ketoacyl-CoA thiolase.
Precautions
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Disclaimer
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