HADHA sirna

HADHA siRNA (Mouse)

Cat. Num. AAA8230266

• Gene Names: Hadha; Mtpa; C77020
• Reactivity: Mouse
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: HADHA; HADHA siRNA (Mouse); Trifunctional enzyme subunit alpha mitochondrial; TP-alpha; HADHA sirna

• Host: Synthetic
• Reactivity: Mouse
• Specificity: HADHA siRNA (Mouse) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 763

• Applicable Applications for HADHA sirna: RNA Interference (RNAi)
• 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 mouse 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.

Related Product Information for HADHA sirna

siRNA to inhibit HADHA expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 33859811
• NCBI GeneID: 97212
• NCBI Accession #: NP_849209.1
• NCBI GenBank Nucleotide #: NM_178878.2
• UniProt Accession #: Q8BMS1; Q3TCY3; Q5U5Y5; Q8QZU4
• Molecular Weight: 82,670 Da
• NCBI Official Full Name: trifunctional enzyme subunit alpha, mitochondrial
• NCBI Official Synonym Full Names: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
• NCBI Official Symbol: Hadha
• NCBI Official Synonym Symbols: Mtpa; C77020
• NCBI Protein Information: trifunctional enzyme subunit alpha, mitochondrial
• UniProt Protein Name: Trifunctional enzyme subunit alpha, mitochondrial
• Protein Family: Trifunctional enzyme
• UniProt Gene Name: Hadha
• UniProt Entry Name: ECHA_MOUSE

Uniprot Description

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: Lipid Metabolism - fatty acid elongation in mitochondria; Secondary Metabolites Metabolism - limonene and pinene degradation; Lipid Metabolism - fatty acid; Mitochondrial; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - propanoate; Lipid Metabolism - unsaturated fatty acid biosynthesis; Acetyltransferase; Carbohydrate Metabolism - butanoate; EC 4.2.1.17; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.1.1.211; Oxidoreductase; Other Amino Acids Metabolism - beta-alanine; Lyase; 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; lyase activity; oxidoreductase activity; acetyl-CoA C-acyltransferase activity; oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor; 3-hydroxyacyl-CoA dehydrogenase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; coenzyme binding; catalytic activity; NAD binding

Biological Process: response to drug; fatty acid beta-oxidation; metabolic process; lipid metabolic process; fatty acid metabolic process; response to insulin stimulus