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HADHA recombinant protein :: Trifunctional enzyme subunit alpha, mitochondrial (HADHA) Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS1108725 HADHA recombinant protein
Unit / Price
0.05 mg (E-Coli)  /  $1,160 +1 FREE 8GB USB
0.05 mg (Yeast)  /  $1,190 +1 FREE 8GB USB
0.05 mg (Baculovirus)  /  $1,305 +1 FREE 8GB USB
0.05 mg (Mammalian-Cell)  /  $1,555 +1 FREE 8GB USB
0.2 mg (E-Coli)  /  $1,565 +1 FREE 8GB USB
0.2 mg (Yeast)  /  $1,610 +2 FREE 8GB USB
0.1 mg (Baculovirus)  /  $1,655 +2 FREE 8GB USB
0.5 mg (E-Coli)  /  $1,725 +2 FREE 8GB USB
0.5 mg (Yeast)  /  $1,825 +2 FREE 8GB USB
0.1 mg (Mammalian-Cell)  /  $2,340 +2 FREE 8GB USB
0.5 mg (Baculovirus)  /  $2,365 +2 FREE 8GB USB
1 mg (E-Coli)  /  $2,460 +3 FREE 8GB USB
1 mg (Yeast)  /  $2,755 +3 FREE 8GB USB
1 mg (Baculovirus)  /  $3,095 +3 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Trifunctional enzyme subunit alpha, mitochondrial (HADHA), Recombinant Protein

 Also Known As   

Recombinant Human Trifunctional enzyme subunit alpha, mitochondrial (HADHA)

 Product Synonym Names    Trifunctional enzyme subunit alpha, mitochondrial; 78 kDa gastrin-binding protein; TP-alpha; Including 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
 Product Gene Name   

HADHA recombinant protein

[Similar Products]
 Product Synonym Gene Name    HADHA; HADH [Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence Positions    37-763aa; full length protein
 Sequence    TRTH INYGVKGDVA VVRINSPNSK VNTLSKELHS EFSEVMNEIW ASDQIRSAVL ISSKPGCFIA GADINMLAAC KTLQEVTQLS QEAQRIVEKL EKSTKPIVAA INGSCLGGGL EVAISCQYRI ATKDRKTVLG TPEVLLGALP GAGGTQRLPK MVGVPAALDM MLTGRSIRAD RAKKMGLVDQ LVEPLGPGLK PPEERTIEYL EEVAITFAKG LADKKISPKR DKGLVEKLTA YAMTIPFVRQ QVYKKVEEKV RKQTKGLYPA PLKIIDVVKT GIEQGSDAGY LCESQKFGEL VMTKESKALM GLYHGQVLCK KNKFGAPQKD VKHLAILGAG LMGAGIAQVS VDKGLKTILK DATLTALDRG QQQVFKGLND KVKKKALTSF ERDSIFSNLT GQLDYQGFEK ADMVIEAVFE DLSLKHRVLK EVEAVIPDHC IFASNTSALP ISEIAAVSKR PEKVIGMHYF SPVDKMQLLE IITTEKTSKD TSASAVAVGL KQGKVIIVVK DGPGFYTTRC LAPMMSEVIR ILQEGVDPKK LDSLTTSFGF PVGAATLVDE VGVDVAKHVA EDLGKVFGER FGGGNPELLT QMVSKGFLGR KSGKGFYIYQ EGVKRKDLNS DMDSILASLK LPPKSEVSSD EDIQFRLVTR FVNEAVMCLQ EGILATPAEG DIGAVFGLGF PPCLGGPFRF VDLYGAQKIV DRLKKYEAAY GKQFTPCQLL ADHANSPNKK FYQ
 OMIM    609016
 3D Structure    ModBase 3D Structure for P40939
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 Host    E Coli or Yeast or Baculovirus or Mammalian Cell
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 Purity/Purification    >=90% (lot specific)
 Form/Format    Liquid containing glycerol
 Tag Information    This protein contains an N-terminal tag and may also contain a C-terminal Myc-tag. N-terminal host tags may vary (His, His-SUMO, His-B2M, GST). Tag types are determined by various factors including tag-protein stability and, therefore, are subject to change; please inquire for tag information.
 Sterility    Sterile filter available upon request.
 Endotoxin    Low endotoxin available upon request.
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 Species    Homo sapiens (Human)
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 Preparation and Storage    Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of HADHA recombinant protein 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.
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Product Description specifically for HADHA recombinant protein

   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.
NCBI/Uniprot data below describe general gene information for HADHA. It may not necessarily be applicable to this product.
 NCBI GI #    20127408
 NCBI GeneID    3030
 NCBI Accession #    NP_000173.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000182.4 [Other Products]
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 UniProt Primary Accession #    P40939 [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
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 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   
GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
[Similar Products]
 NCBI Protein Information    trifunctional enzyme subunit alpha, mitochondrial; 3-oxoacyl-CoA thiolase; gastrin-binding protein; 78 kDa gastrin-binding protein; long-chain 2-enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
 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
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 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]
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 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 - propanoate; Amino Acid Metabolism - tryptophan; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase; EC 1.1.1.211; Lipid Metabolism - unsaturated fatty acid biosynthesis; Amino Acid Metabolism - lysine degradation; Acetyltransferase; Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.17; Carbohydrate Metabolism - butanoate; Secondary Metabolites Metabolism - limonene and pinene degradation; Lipid Metabolism - fatty acid elongation in mitochondria

Chromosomal Location of Human Ortholog: 2p23

Cellular Component: mitochondrion; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex

Molecular Function: protein binding; acetyl-CoA C-acetyltransferase activity; 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; 3-hydroxyacyl-CoA dehydrogenase activity

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

Disease: Trifunctional Protein Deficiency; Long-chain 3-hydroxyacyl-coa Dehydrogenase Deficiency
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 Research Articles on HADHA    1. Report probable Kashubian origin of the prevalent HADHA c.1528G>C mutation responsible for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency in Poland.
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 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.
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Products associated with HADHA recombinant proteinPathways associated with HADHA recombinant protein
 Reference Product  PubMed Publications
 HADHB recombinant protein  >13 publications with HADHA and HADHB
 ACADL recombinant protein  >1 publications with HADHA and ACADL
 ACADVL recombinant protein  >1 publications with HADHA and ACADVL
 ACAA2 recombinant protein  >1 publications with HADHA and ACAA2
 Products by Pathway  Pathway Diagram
 Acyl Chain Remodeling Of CL Pathway antibodies  Acyl Chain Remodeling Of CL Pathway Diagram
 Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway antibodies  Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway Diagram
 Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway antibodies  Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway Diagram
 Beta Oxidation Of Lauroyl-CoA To Decanoyl-CoA-CoA Pathway antibodies  Beta Oxidation Of Lauroyl-CoA To Decanoyl-CoA-CoA Pathway Diagram
 Beta Oxidation Of Myristoyl-CoA To Lauroyl-CoA Pathway antibodies  Beta Oxidation Of Myristoyl-CoA To Lauroyl-CoA Pathway Diagram
 Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway antibodies  Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway Diagram
 Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway antibodies  Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway Diagram
 Biosynthesis Of Unsaturated Fatty Acids Pathway antibodies  Biosynthesis Of Unsaturated Fatty Acids Pathway Diagram
 Biosynthesis Of Unsaturated Fatty Acids Pathway antibodies  Biosynthesis Of Unsaturated Fatty Acids Pathway Diagram
 Butanoate Metabolism Pathway antibodies  Butanoate Metabolism Pathway Diagram
Diseases associated with HADHA recombinant proteinOrgans/Tissues associated with HADHA recombinant protein
 Disease Name  Pubmed Publications
 LONG-CHAIN 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY Antibodies  >14 publications with HADHA and LONG-CHAIN 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY
 Trifunctional Protein Deficiency Antibodies  >8 publications with HADHA and Trifunctional Protein Deficiency
 Fatty Liver Antibodies  >6 publications with HADHA and Fatty Liver
 Mitochondrial Diseases Antibodies  >5 publications with HADHA and Mitochondrial Diseases
 Liver Diseases Antibodies  >3 publications with HADHA and Liver Diseases
 Cardiovascular Diseases Antibodies  >2 publications with HADHA and Cardiovascular Diseases
 Nervous System Diseases Antibodies  >2 publications with HADHA and Nervous System Diseases
 Drug Toxicity Antibodies  >2 publications with HADHA and Drug Toxicity
 Carcinoma Antibodies  >2 publications with HADHA and Carcinoma
 Liver Failure, Acute Antibodies  >2 publications with HADHA and Liver Failure, Acute
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >6 publications with HADHA and Blood
 Liver Antibodies  >6 publications with HADHA and Liver
 Muscle Antibodies  >6 publications with HADHA and Muscle
 Heart Antibodies  >4 publications with HADHA and Heart
 Eye Antibodies  >1 publications with HADHA and Eye
 Placenta Antibodies  >1 publications with HADHA and Placenta
 Skin Antibodies  >1 publications with HADHA and Skin
 Lung Antibodies  >1 publications with HADHA and Lung
 Uterus Antibodies  >1 publications with HADHA and Uterus
 Adipose Tissue Antibodies  >1 publications with HADHA and Adipose Tissue
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