Product Name
HADHA, cDNA Clone
Full Product Name
HADHA cDNA Clone
Product Gene Name
HADHA cdna clone
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
atggtggcct gccgggcgat tggcatcctc agccgctttt ctgccttcag gatcctccgc tcccgaggtt atatatgccg caattttaca gggtcttctg ctttgctgac cagaacccat attaactatg gagtcaaagg ggatgtggca gttgttcgaa ttaactctcc caattcaaag gtaaatacac tgagtaaaga gctacattca gagttctcag aagttatgaa tgaaatctgg gctagtgatc aaatcagaag tgccgtcctt atctcatcaa agccaggctg ctttattgca ggtgctgata tcaacatgtt agccgcttgc aagacccttc aagaagtaac acagctatca caagaagcac agagaatagt tgagaaactt gaaaagtcca caaagcctat tgtggctgcc atcaatggat cctgcctggg aggaggactt gaggttgcca tttcatgcca atacagaata gcaacaaaag acagaaaaac agtattaggt acccctgaag ttttgctggg ggccttacca ggagcaggag gcacacaaag gctgcccaaa atggtgggtg tgcctgctgc tttggacatg atgctgactg gtagaagcat tcgtgcagac agggcaaaga aaatgggact ggttgaccaa ctggtggaac ccctgggacc aggactaaaa cctccagagg aacggacaat agaataccta gaagaagttg caattacttt tgccaaagga ctagctgata agaagatctc tccaaagaga gacaagggat tggtggaaaa attgacagcg tatgccatga ctattccatt tgtcaggcaa caggtttaca aaaaagtgga agaaaaagtg cgaaagcaga ctaaaggcct ttatcctgca cctctgaaaa taattgatgt ggtaaagact ggaattgagc aagggagtga tgccggttat ctctgtgaat ctcagaaatt tggagagctt gtaatgacca aagaatcaaa ggccttgatg ggactctacc atggtcaggt cctgtgcaag aagaataaat ttggagctcc acagaaggat gttaagcatc tggctattct tggtgcaggg ctgatgggag caggcatcgc ccaagtctcc gtggataagg ggctaaagac tatacttaaa gatgccaccc tcactgcgct agaccgagga cagcaacaag tgttcaaagg attgaatgac aaagtgaaga agaaagctct aacatcattt gaaagggatt ccatcttcag caacttgact gggcagcttg attaccaagg ttttgaaaag gccgacatgg tgattgaagc tgtgtttgag gaccttagtc ttaagcacag agtgctaaag gaagtagaag cggtgattcc agatcactgt atctttgcca gtaacacatc tgctctccca atcagtgaaa tcgctgctgt cagcaaaaga cctgagaagg tgattggcat gcactacttc tctcccgtgg acaagatgca gctgctggag attatcacga ccgagaaaac ttccaaagac accagtgctt cagctgtagc agttggtctc aagcagggga aggtcatcat tgtggttaag gatggacctg gcttctatac taccaggtgt cttgcgccca tgatgtctga agtcatccga atcctccagg aaggagttga cccgaagaag ctggattccc tgaccacaag ctttggcttt cctgtgggtg ccgccacact ggtggatgaa gttggtgtgg atgtagcgaa acatgtggcg gaagatctgg gcaaagtctt tggggagcgg tttggaggtg gaaacccaga actgctgaca cagatggtgt ccaagggctt cctaggtcgt aaatctggga agggctttta catctatcag gagggtgtga agaggaagga tttgaattct gacatggata gtattttagc gagtctgaag ctgcctccta agtctgaagt ctcatcagac gaagacatcc agttccgcct ggtgacaaga tttgtgaatg aggcagtcat gtgcctgcaa gaggggatct tggccacacc tgcagaggga gacatcggag ccgtctttgg gcttggcttc ccgccttgtc tgggagggcc tttccgcttt gtggatctgt atggcgccca gaagatagtg gaccggctca agaaatatga agctgcctat ggaaaacagt tcaccccatg ccagctgcta gctgaccatg ctaacagccc taacaagaag ttctaccagt ga
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of HADHA cdna clone 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.
NCBI/Uniprot data below describe general gene information for HADHA. It may not necessarily be applicable to this product.
NCBI Accession #
BC009235
[Other Products]
UniProt Secondary Accession #
Q16679; Q53T69; Q53TA2; Q96GT7; Q9UQC5; B2R7L4; B4DYP2[Other Products]
UniProt Related Accession #
P40939[Other Products]
Molecular Weight
28,367 Da
NCBI Official Full Name
Homo sapiens hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit, mRNA
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
GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
[Similar Products]
NCBI Protein Information
trifunctional enzyme subunit alpha, mitochondrial
UniProt Protein Name
Trifunctional enzyme subunit alpha, mitochondrial
UniProt Synonym Protein Names
78 kDa gastrin-binding protein; 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 Synonym Gene Names
HADH [Similar Products]
UniProt Entry Name
ECHA_HUMAN
NCBI Summary for HADHA
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]
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: Carbohydrate Metabolism - butanoate; EC 1.1.1.211; Acetyltransferase; Mitochondrial; Lyase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.17; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid elongation in mitochondria; Oxidoreductase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - tryptophan; Secondary Metabolites Metabolism - limonene and pinene degradation; Lipid Metabolism - unsaturated fatty acid biosynthesis; Amino Acid Metabolism - lysine degradation
Chromosomal Location of Human Ortholog: 2p23
Cellular Component: extracellular matrix; mitochondrial inner membrane; mitochondrion
Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; acetyl-CoA C-acetyltransferase activity; protein binding
Biological Process: fatty acid beta-oxidation
Disease: Long-chain 3-hydroxyacyl-coa Dehydrogenase Deficiency; Trifunctional Protein Deficiency
Research Articles on HADHA
1. findings suggest an auxiliary role for HADHA in miRNA biogenesis and help in better understanding of molecular mechanisms underlying RNAi pathway
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
